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GLUTARIC ACID ( GLUTARİK ASİT )

GLUTARIC ACID

SYNONYMS: glutaric acid; glutarik asit; gluterik asit; glutarik asid; gluteric asit; glutaric asit; glutaric acit;GLUTARIC ACID; GLUTARIK ASIT; GLUTARİK ASİT; GLUTARİK ASİD; GLUTERİC ASİT; GLUTARİC ACİT; glutaric acid, calcium salt; glutaric acid, copper(2+) salt (1:1); glutaric acid, disodium salt; glutaric acid, ion(1-); glutaric acid, monosodium salt; Pentanedioic acid; 110-94-1; 1,5-Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentandioic acid; glutarate; n-Pyrotartaric acid; UNII-H849F7N00B; propane-1,3-dicarboxylic acid; Carboxylic acids, di-, C4-6; Glutaric acid, 99%; HSDB 5542; Carboxylic acids, C6-18 and C5-15-di-; NSC 9238; EINECS 203-817-2; MFCD00004410; (C4-C6) Dibasic acids; BRN 1209725; AI3-24247; CHEBI:17859; NSC9238; JFCQEDHGNNZCLN-UHFFFAOYSA-N; H849F7N00B; DSSTox_CID_1654; DSSTox_RID_76266; DSSTox_GSID_21654; CAS-110-94-1; Glutarsaeure; Pentandioate; 1czc; 1,5-Pentanedioate; EINECS 273-081-5; PubChem18989; 4lh3; 1,3-Propanedicarboxylate; 1,3-Propanedicarboxylic acid ChEBI; 1,5-Pentanedioic acid ChEBI; Glutarsaeure ChEBI; Pentanedioic acid ChEBI; 1,3-Propanedicarboxylate Generator; 1,5-Pentanedioate Generator; Pentanedioate Generator; Glutarate Generator; Pentandioate; Glutaric acid Pentanedioic acid 110-94-1 1,5-Pentanedioic acid 1,3-Propanedicarboxylic acid; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide;glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide;glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide;glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; 1,3-Propanedicarboxylic acid; 1,5-Pentanedioic acid; pentanedioic acid; pentanedioate; 1,3-Propanedicarboxylic acid; 1,5-Pentanedioic acid; pentanedioic acid; pentanedioate; 1,3-Propanedicarboxylic acid; 1,5-Pentanedioic acid; pentanedioic acid; pentanedioate; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide;glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide;glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide;glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; 1,3-Propanedicarboxylic acid; 1,5-Pentanedioic acid; pentanedioic acid; pentanedioate; 1,3-Propanedicarboxylic acid; 1,5-Pentanedioic acid; pentanedioic acid; pentanedioate; 1,3-Propanedicarboxylic acid; 1,5-Pentanedioic acid; pentanedioic acid; pentanedioate; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide;glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide;glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide;glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acit; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; glutaric acid; glatarıc asit; gulutaric asit; grutarıc acide; glutarıc acıde; GLUTARIC ACIDE; GLUTARİDE ACİT; GLUTARİT ASİT; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; gılutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARİK ASİT; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARİC ACİT; GLUTARAL ASİT; GLUTARİK ASİT; DİCARBOXYL ASİT; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARİK ASİT ANHİDRİT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAİK ASİT; PENTANEDOİK ASİT; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARİK ASİDÜTE; glutarik asidide; 1,3-Propanedicarboxylic acid; 1,5-Pentanedioic acid; pentanedioic acid; pentanedioate; 1,3-Propanedicarboxylic acid; 1,5-Pentanedioic acid; pentanedioic acid; pentanedioate; 1,3-Propanedicarboxylic acid; 1,5-Pentanedioic acid; pentanedioic acid; pentanedioate;

 


Glutaric acid is a simple five-carbon linear dicarboxylic acid. Glutaric acid is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Glutaric acid may cause irritation to the skin and eyes. When present in sufficiently high levels, glutaric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of glutaric acid are associated with at least three inborn errors of metabolism, including glutaric aciduria type I, malonyl-CoA decarboxylase deficiency, and glutaric aciduria type III. Glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1. 3. 99. 7, GCDH). Excessive levels of their intermediate breakdown products (e. g. glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid, glutaconic acid) can accumulate and cause damage to the brain (and also other organs). Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Macrocephaly is amongst the earliest signs of GA1. GA1 also causes secondary carnitine deficiency because glutaric acid, like other organic acids, is detoxified by carnitine. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7. 35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated glutaric aciduria. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. Treatment of glutaric aciduria is mainly based on the restriction of lysine intake, supplementation of carnitine, and an intensification of therapy during intercurrent illnesses. The major principle of dietary treatment is to reduce the production of glutaric acid and 3-hydroxyglutaric acid by restriction of natural protein, in general, and of lysine, in particular (PMID: 17465389, 15505398).

 

from Human Metabolome Database (HMDB) 1Structures HelpNew Window 1.12D Structure HelpNew Window Find Similar Structures Get Image Download Chemical Structure Depiction Glutaric acid.png Full screen Zoom in Zoom out from PubChem COMPOUND SUMMARY Glutaric acid PubChem CID: 743 Structure: Glutaric acid_small.png Glutaric acid_3D_Structure.png Glutaric acid_Crystal_Structure.png Find Similar Structures Chemical Safety: Corrosive Laboratory Chemical Safety Summary (LCSS) Datasheet Molecular Formula: C5H8O4 or COOH(CH2)3COOH Chemical Names: GLUTARIC ACID Pentanedioic acid 110-94-1 1,5-Pentanedioic acid 1,3-Propanedicarboxylic acid More... Molecular Weight: 132.11 g/mol Dates: Modify: 2019-08-03 Create: 2004-09-16 Glutaric acid is an alpha,omega-dicarboxylic acid that is a linear five-carbon dicarboxylic acid. It has a role as a human metabolite and a Daphnia magna metabolite. It is a conjugate acid of a glutarate(1-) and a glutarate. from ChEBI

Glutaric acid is a simple five-carbon linear dicarboxylic acid. Glutaric acid is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Glutaric acid may cause irritation to the skin and eyes. When present in sufficiently high levels, glutaric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of glutaric acid are associated with at least three inborn errors of metabolism, including glutaric aciduria type I, malonyl-CoA decarboxylase deficiency, and glutaric aciduria type III. Glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1. 3. 99. 7, GCDH). Excessive levels of their intermediate breakdown products (e. g. glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid, glutaconic acid) can accumulate and cause damage to the brain (and also other organs). Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Macrocephaly is amongst the earliest signs of GA1. GA1 also causes secondary carnitine deficiency because glutaric acid, like other organic acids, is detoxified by carnitine. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7. 35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated glutaric aciduria. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. Treatment of glutaric aciduria is mainly based on the restriction of lysine intake, supplementation of carnitine, and an intensification of therapy during intercurrent illnesses. The major principle of dietary treatment is to reduce the production of glutaric acid and 3-hydroxyglutaric acid by restriction of natural protein, in general, and of lysine, in particular (PMID: 17465389, 15505398).

Glutaric acid is a simple five-carbon linear dicarboxylic acid. Glutaric acid is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Glutaric acid may cause irritation to the skin and eyes. When present in sufficiently high levels, glutaric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of glutaric acid are associated with at least three inborn errors of metabolism, including glutaric aciduria type I, malonyl-CoA decarboxylase deficiency, and glutaric aciduria type III. Glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1. 3. 99. 7, GCDH). Excessive levels of their intermediate breakdown products (e. g. glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid, glutaconic acid) can accumulate and cause damage to the brain (and also other organs). Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Macrocephaly is amongst the earliest signs of GA1. GA1 also causes secondary carnitine deficiency because glutaric acid, like other organic acids, is detoxified by carnitine. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7. 35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated glutaric aciduria. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. Treatment of glutaric aciduria is mainly based on the restriction of lysine intake, supplementation of carnitine, and an intensification of therapy during intercurrent illnesses. The major principle of dietary treatment is to reduce the production of glutaric acid and 3-hydroxyglutaric acid by restriction of natural protein, in general, and of lysine, in particular (PMID: 17465389, 15505398).

 

2.3.7Wikipedia HelpNew Window
Glutaric acid

 

glutaric acid, calcium salt glutaric acid, copper(2+) salt (1:1) glutaric acid, disodium salt glutaric acid, ion(1-) glutaric acid, monosodium salt from MeSH 2.4.2Depositor-Supplied Synonyms HelpNew Window GLUTARIC ACID Pentanedioic acid 110-94-1 1,5-Pentanedioic acid 1,3-Propanedicarboxylic acid Pentandioic acid glutarate n-Pyrotartaric acid UNII-H849F7N00B propane-1,3-dicarboxylic acid Carboxylic acids, di-, C4-6 Glutaric acid, 99% HSDB 5542 Carboxylic acids, C6-18 and C5-15-di- NSC 9238 EINECS 203-817-2 MFCD00004410 (C4-C6) Dibasic acids BRN 1209725 AI3-24247 CHEBI:17859 NSC9238 JFCQEDHGNNZCLN-UHFFFAOYSA-N H849F7N00B DSSTox_CID_1654 DSSTox_RID_76266 DSSTox_GSID_21654 CAS-110-94-1 Glutarsaeure Pentandioate 1czc 1,5-Pentanedioate EINECS 273-081-5 PubChem18989 4lh3 1,3-Propanedicarboxylate WLN: QV3VQ Glutaric acid From Wikipedia, the free encyclopedia Jump to navigationJump to search Not to be confused with Glutamic acid. Glutaric acid Skeletal formula of glutaric acid Ball-and-stick model of the glutaric acid molecule Names Preferred IUPAC name Pentanedioic acid Other names Glutaric acid Propane-1,3-dicarboxylic acid 1,3-Propanedicarboxylic acid Pentanedioic acid n-Pyrotartaric acid Identifiers CAS Number 110-94-1 ☑ 3D model (JSmol) Interactive image ChEBI :17859 ☑ ChEMBL ChEMBL1162495 ☑ ChemSpider 723 ☑ DrugBank DB03553 ☑ ECHA InfoCard 100.003.471 EC Number 203-817-2 KEGG C00489 ☑ PubChem CID 743 CompTox Dashboard (EPA) DTXSID2021654 Edit this at Wikidata InChI SMILES Properties Chemical formula C5H8O4 Molar mass 132.12 g/mol Melting point 95 to 98 °C (203 to 208 °F; 368 to 371 K) Boiling point 200 °C (392 °F; 473 K) /20 mmHg Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa). ☑ verify (what is ☑☒ ?) Infobox references Glutaric acid is the organic compound with the formula C3H6(COOH)2 . Although the related "linear" dicarboxylic acids adipic and succinic acids are water-soluble only to a few percent at room temperature, the water-solubility of glutaric acid is over 50% (w/w).

 


Contents 1 Biochemistry 2 Production 3 Uses 4 Safety 5 References 6 External links Biochemistry Glutaric acid is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Defects in this metabolic pathway can lead to a disorder called glutaric aciduria, where toxic byproducts build up and can cause severe encephalopathy.

 

 

Production
Glutaric acid can be prepared by the ring-opening of butyrolactone with potassium cyanide to give the mixed potassium carboxylate-nitrile that is hydrolyzed to the diacid.[1] Alternatively hydrolysis, followed by oxidation of dihydropyran gives glutaric acid. It can also be prepared from reacting 1,3-dibromopropane with sodium or potassium cyanide to obtain the dinitrile, followed by hydrolysis.

 

 

Uses
1,5-Pentanediol, a common plasticizer and precursor to polyesters is manufactured by hydrogenation of glutaric acid and its derivatives.[2]
Glutaric acid itself has been used in the production of polymers such as polyester polyols, polyamides. The odd number of carbon atoms (i.e. 5) is useful in decreasing polymer elasticity.[citation needed]
Uvitonic acid is obtained by the action of ammonia on glutaric acid.
Safety
Glutaric acid may cause irritation to the skin and eyes.[3] Acute hazards include the fact that this compound may be harmful by ingestion, inhalation or skin absorption.[3]

 

Glutaric acid is a simple five-carbon linear dicarboxylic acid. Glutaric acid is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Glutaric acid may cause irritation to the skin and eyes. When present in sufficiently high levels, glutaric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of glutaric acid are associated with at least three inborn errors of metabolism, including glutaric aciduria type I, malonyl-CoA decarboxylase deficiency, and glutaric aciduria type III. Glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH). Excessive levels of their intermediate breakdown products (e.g. glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid, glutaconic acid) can accumulate and cause damage to the brain (and also other organs). Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Macrocephaly is amongst the earliest signs of GA1. GA1 also causes secondary carnitine deficiency because glutaric acid, like other organic acids, is detoxified by carnitine. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated glutaric aciduria. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. Treatment of glutaric aciduria is mainly based on the restriction of lysine intake, supplementation of carnitine, and an intensification of therapy during intercurrent illnesses. The major principle of dietary treatment is to reduce the production of glutaric acid and 3-hydroxyglutaric acid by restriction of natural protein, in general, and of lysine, in particular (PMID: 17465389 , 15505398 ). Glutaric acid has also been found in Escherichia (PMID: 30143200 ).

Synonyms Value Source 1,3-Propanedicarboxylic acid ChEBI 1,5-Pentanedioic acid ChEBI Glutarsaeure ChEBI Pentanedioic acid ChEBI 1,3-Propanedicarboxylate Generator 1,5-Pentanedioate Generator Pentanedioate Generator Glutarate Generator Pentandioate HMDB Pentandioic acid HMDB Chemical Formula C5H8O4 Average Molecular Weight 132.1146 Monoisotopic Molecular Weight 132.042258744 IUPAC Name pentanedioic acid Traditional Name glutaric acid Biospecimen Status Value Age Sex Condition Reference Details Blood Detected and Quantified 12 uM Newborn (0-30 days old) Male Glutaric aciduria I 9350903 Cerebrospinal Fluid (CSF) Detected and Quantified 39.74 uM Not Available Not Specified Glutaryl-CoA dehydrogenase deficiency (GDHD) 10604139 Cerebrospinal Fluid (CSF) Detected and Quantified 43 uM Newborn (0-30 days old) Male Glutaric aciduria I 9350903 Feces Detected but not Quantified Adult (>18 years old) Both Irritable bowel syndrome 21761941 Feces Detected but not Quantified Adult (>18 years old) Both Ulcerative colitis 21761941 Feces Detected but not Quantified Not Specified Not Specified Cryptosporidium infection 22944170 Feces Detected but not Quantified Adult (>18 years old) Both Colorectal cancer 25037050 Feces Detected but not Quantified Adult (>18 years old) Both Colorectal Cancer 27275383

 

Disease References 
Glutaric aciduria I
Poge AP, Autschbach F, Korall H, Trefz FK, Mayatepek E: Early clinical manifestation of glutaric aciduria type I and nephrotic syndrome during the first months of life. Acta Paediatr. 1997 Oct;86(10):1144-7. [PubMed:9350903 ]
Fraidakis MJ, Liadinioti C, Stefanis L, Dinopoulos A, Pons R, Papathanassiou M, Garcia-Villoria J, Ribes A: Rare Late-Onset Presentation of Glutaric Aciduria Type I in a 16-Year-Old Woman with a Novel GCDH Mutation. JIMD Rep. 2015;18:85-92. doi: 10.1007/8904_2014_353. Epub 2014 Sep 26. [PubMed:25256449 ]
G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Glutaryl-CoA dehydrogenase deficiency (GDHD)
Baric I, Wagner L, Feyh P, Liesert M, Buckel W, Hoffmann GF: Sensitivity and specificity of free and total glutaric acid and 3-hydroxyglutaric acid measurements by stable-isotope dilution assays for the diagnosis of glutaric aciduria type I. J Inherit Metab Dis. 1999 Dec;22(8):867-81. [PubMed:10604139 ]
Irritable bowel syndrome

 

 

Glutaric aciduria type III
Knerr I, Zschocke J, Trautmann U, Dorland L, de Koning TJ, Muller P, Christensen E, Trefz FK, Wundisch GF, Rascher W, Hoffmann GF: Glutaric aciduria type III: a distinctive non-disease? J Inherit Metab Dis. 2002 Oct;25(6):483-90. [PubMed:12555941 ]
Jakobs C, Sweetman L, Wadman SK, Duran M, Saudubray JM, Nyhan WL: Prenatal diagnosis of glutaric aciduria type II by direct chemical analysis of dicarboxylic acids in amniotic fluid. Eur J Pediatr. 1984 Jan;141(3):153-7. [PubMed:6698061 ]
Whelan DT, Hill R, Ryan ED, Spate M: L-Glutaric acidemia: investigation of a patient and his family. Pediatrics. 1979 Jan;63(1):88-93. [PubMed:440804 ]
Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed:8412012 ]
Singh I: Biochemistry of peroxisomes in health and disease. Mol Cell Biochem. 1997 Feb;167(1-2):1-29. [PubMed:9059978 ]
Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
Bishop FS, Liu JK, McCall TD, Brockmeyer DL: Glutaric aciduria type 1 presenting as bilateral subdural hematomas mimicking nonaccidental trauma. Case report and review of the literature. J Neurosurg. 2007 Mar;106(3 Suppl):222-6. [PubMed:17465389 ]
Muller E, Kolker S: Reduction of lysine intake while avoiding malnutrition--major goals and major problems in dietary treatment of glutaryl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 2004;27(6):903-10. [PubMed:15505398 ]
Hong YG, Moon YM, Hong JW, No SY, Choi TR, Jung HR, Yang SY, Bhatia SK, Ahn JO, Park KM, Yang YH: Production of glutaric acid from 5-aminovaleric acid using Escherichia coli whole cell bio-catalyst overexpressing GabTD from Bacillus subtilis. Enzyme Microb Technol. 2018 Nov;118:57-65. doi: 10.1016/j.enzmictec.2018.07.002. 
Kobayashi Y, Ohshiro N, Shibusawa A, Sasaki T, Tokuyama S, Sekine T, Endou H, Yamamoto T: Isolation, characterization and differential gene expression of multispecific organic anion transporter 2 in mice. Mol Pharmacol. 2002 Jul;62(1):7-14. [PubMed:12065749 ]
Glutaric acid

 

 

Glutaric acid for synthesis. CAS 110-94-1, EC Number 203-817-2, chemical formula HOOC(CH₂)₃COOH.
Glutaric acid: Malzeme Güvenlik Bilgi Formu (MSDS) veya SDS, Analiz Sertifikası (COA) ve Kalite Uygunluk Sertifikası (COQ), dosyalar, broşürler ve diğer dokümanlar.

 

 

Glutaric acid
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Glutaric acid
99%
CAS Number 110-94-1 Linear Formula HOOC(CH2)3COOH Molecular Weight 132.11 Beilstein/REAXYS Number 1209725 EC Number 203-817-2 MDL number MFCD00004410 PubChem Substance ID 24895125
Glutaric acid 99%
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Purchase Safety & Documentation Protocols & Articles3 Peer-Reviewed Papers66
Properties
Related Categories Building Blocks, C1 to C5, Carbonyl Compounds, Carboxylic Acids, Chemical Synthesis,
More...
InChI Key JFCQEDHGNNZCLN-UHFFFAOYSA-N
assay 99%
bp 200 °C/20 mmHg(lit.)
mp 95-98 °C (lit.)
solubility water: soluble 5 mg/mL, clear to slightly hazy, colorless to faintly yellow
alcohol: soluble(lit.)
chloroform: soluble(lit.)
Description
Packaging
25, 100, 500 g in poly bottle

 

 

Application
Glutaric acid may be employed as starting reagent in the synthesis of glutaric anhydride.[9]

 

 

Glutaric acid may be used for the following studies:
• Complexation with DL-lysine. Complexes have been reported to possess zwitterionic lysinium ions (positively charged) and semi-glutarate ions (negatively charged).[8]
• Synthesis of complexes with L-arginine and L-histidine.[7]
• Preparation of glycine-glutaric acid co-crystals. Phase transition studies of these cocrystals have been reported by single-crystal X-ray diffraction, polarized Raman spectroscopy and differential scanning calorimetry.[1]
General description
Glutaric acid (Pentanedioic Acid) is a linear dicarboxylic acid. It has been prepared by oxidizing cyclopentane, cyclopentanol and cyclopentanone.[9]

 

Glutaric acid is a pentanedioic acid. On exposure to X-rays, glutaric acid crystals generate two stable free radicals. These free radicals have been investigated by electron nuclear double resonance (ENDOR) technique.[5] Presence of glutaric acid in urine and plasma is an indicator of type I glutaric aciduria (GA-I).[6]

Glutaric acid is formed as an intermediate during the catabolism of lysine in mammals.[3] Electron spin resonance spectra of radical (CO2H)CH2CH2CH(CO2H formed in glutaric acid crystal after γ-irradiation is reported to remains trapped in it.[2] Polymorphism of Glycine-glutaric acid co-crystals has been studied by single crystal X-ray diffraction and Raman spectroscopy.[4]

 

Other Notes
Tandem Mass Spectrometry data independently generated by Scripps Center for Metabolomics is available to view or download in PDF. G3407.pdf Tested metabolites are featured on Scripps Center for Metabolomics METLIN Metabolite Database. To learn more, visit sigma.com/metlin.

 

 

Safety Information
Symbol GHS07 GHS07 Signal word Warning Hazard statements H319 Precautionary statements P305 + P351 + P338 Personal Protective Equipment dust mask type N95 (US), Eyeshields, Gloves RIDADR NONH for all modes of transport WGK Germany 1 RTECS MA3740000
Documents
Certificate of Analysis

 

 

Meet Synthia - Retrosynthesis Sofware
Protocols & Articles
Articles
Characteristic Metabolites for Inborn Errors of Amino Acid Metabolism
Inborn errors of metabolism are caused by changes in specific enzymatic reactions and hundreds of different such alterations, which affect about 1 of every 5000 newborns, have been characterized. The...
Keywords: Indicators, Metabolism, Metabolites

 

 

MSMLS Online Plate Map
Metabolite descriptors included with the software download upon purchase of the product include: Name, Parent CID, molecular formula, molecular weight, CAS, ChEBI, HMDB ID, PubChem Compound and Subst...
Keywords: Mass spectrometry, Metabolites

 

 

OAMLS Online Plate Map
OAMLS compounds are conveniently provided at 5µg per well, enough for multiple injections. These are high purity (>95%) compounds supplied in an economical, ready to use format. Compounds are arrange...
Keywords: Metabolites

 

 

Peer-Reviewed Papers
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Read Abstract 
Low-temperature phase transition in glycine-glutaric acid co-crystals studied by single-crystal X-ray diffraction, Raman spectroscopy and differential scanning calorimetry.
Boris A Zakharov et. al
Acta crystallographica. Section B, Structural science, 68(Pt 3), undefined (2012-5-23)
The occurrence of a first-order reversible phase transition in glycine-glutaric acid co-crystals at 220-230 K has been confirmed by three different techniques - single-crystal X-ray diffraction, polarized Raman spectroscopy and differential scanning ...Read More
Electron spin resonance of (CO2H) CH2CH2CH (CO2H) in irradiated glutaric acid. Horsfield A, et al. Molecular Physics 4(2), 169-175, (1961)
Read Abstract 
The metabolism of glutaric acid-3-C14 by the intact rat.
D C HOBBS and R E KOEPPE
The Journal of biological chemistry, 230(2), undefined (1958-2-1)
Polymorphism of ?glycine-glutaric acid? co-crystals: the same phase at low temperatures and high pressures. Zakharov BA, et al. CrystEngComm 15(9), 1693-1697, (2013)
ENDOR Studies in Molecular Crystals. I. Glutaric Acid at 4.2? K. Kwiram AL. J. Chem. Phys. 55(5), 2484-2495, (1971)
Read Abstract 
Glutaric aciduria type I: a common cause of episodic encephalopathy and spastic paralysis in the Amish of Lancaster County, Pennsylvania.
D H Morton et. al
American journal of medical genetics, 41(1), undefined (1991-10-1)
We have diagnosed type I glutaric aciduria (GA-I) in 14 children from 7 Old Order Amish families in Lancaster County, Pennsylvania. An otherwise rare disorder, GA-I appears to be a common cause of acute encephalopathy and cerebral palsy among the Ami...Read More
Read Abstract 
X-ray studies on crystalline complexes involving amino acids and peptides. XXXVIII. Crystal structures of the complexes of L-arginine and L-histidine with glutaric acid and a comparative study of amino acid-glutaric acid complexes.
N T Saraswathi and M Vijayan
Acta crystallographica. Section B, Structural science, 57(Pt 6), undefined (2001-11-22)
The complexes of glutaric acid with L-arginine and L-histidine (two crystal forms) exhibit different stoichiometries and ionization states. The aggregation patterns in two of the crystals are remarkably similar to those observed earlier in similar st...Read More
X-ray studies on crystalline complexes involving amino acids and peptides. XXXVII. Novel aggregation patterns and effect of chirality in the complexes of DL-and L-lysine with glutaric acid. Saraswathi NT, et al. Acta Crystallographica Section B, Structural Science, Crystal Engineering and Materials 53(1), 366-371, (2001)
Eagleson M. Concise Encyclopedia Chemistry , 461-461, (1994)
Read Abstract 
Prenatal diagnosis of glutaric aciduria type II by direct chemical analysis of dicarboxylic acids in amniotic fluid.
C Jakobs et. al
European journal of pediatrics, 141(3), undefined (1984-1-1)
A method for the measurement of dicarboxylic acids in amniotic fluid was developed that utilizes isolation of the acids by liquid partition chromatography and quantification by ammonia chemical ionization selected ion monitoring, gas chromatography-m...Read More
Read Abstract 
Antenatal diagnosis of glutaric acidemia.
S I Goodman et. al
American journal of human genetics, 32(5), undefined (1980-9-1)
Two pregnancies at risk for glutaric acidemia were monitored. In one, in which the fetus was not affected, glutaric acid was not detected in the amniotic fluid at amniocentesis (15 weeks) and the glutaryl-CoA dehydrogenase activity of cultured amniot...Read More
Read Abstract 
L-Glutaric acidemia: investigation of a patient and his family.
D T Whelan et. al
Pediatrics, 63(1), undefined (1979-1-1)
A 5-month-old infant had an unusual combination of clinical signs and symptoms. These consisted of irritability, dystonia, lack of head control, grimacing, opisthotonos, choreoathetoid movements, delayed development, and severe metabolic acidosis. Me...Read More

 

The significant problem with the glutaric acid formulation is the resulting predictions for impurity RRT 1.22. The model shown in Fig. 4 shows a decelerating model of moderate temperature dependence (Ea/R or K2 = 9000 K), but no significant humidity sensitivity (N1 = 0.02). The formation of RRT 1.22 was best modeled with the "Decelerating Kinetic Model," which is a model type typically associated with the presence of a limiting reagent or limiting equilibrium. The problem with exponential decelerating behavior is the initial period of impurity formation is rapid, and it exponentially slows down as it reaches an upper limit. The total impurity formation that could happen is approximately 0.7% (defined as C + K3 in the model), which is well above specification limits of 0.2%.

 


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Fig. 4. Model evaluation for the glutaric acid formulation RRT 1.22 impurity (a screenshot from the ASM software).

 

Extreme measures would be needed to prevent the rapid approach toward 0.7% RRT 1.22 content, with no capability to control via desiccation or packaging selection. The resulting model predictions shown in Fig. 5 indicate the only option would be frozen storage at - 20°C to achieve a shelf life of 2 years. Since this is a less desirable outcome than can be achieved with the lactic acid formulation, there is no need to further consider the glutaric acid formulation in this analysis, and the lactic acid formulation was selected for further development.

 

Glutaric acid
Glutaric aciduria type 1 (OMIM #231670) due to glutaryl-coenzyme A dehydrogenase deficiency is associated with accumulation of glutaric acid, glutaryl carnitine, and secondary metabolites in body fluids. The clinical picture is variable. Most patients are macrocephalic. A large proportion present between the first weeks and the sixth year of life with an acute encephalopathy, usually resulting in severe permanent dystonia. As this evolution can be prevented (by carnitine supplementation and dietary restriction), early presymptomatic recognition is of utmost importance. Therefore, many countries have introduced neonatal screening programs. Rarely, patients have a milder phenotype and present with ataxia. Almost all patients can be diagnosed by urine analysis for organic acids (with the rare exception of "low secretors"), suggesting that an acylcarnitine profile in dried blood spots is perhaps more reliable.

 

Compound 1 is a poorly soluble drug with <0.1 µg/mL in aqueous media. Salt formation was not possible even with strong acids such as hydrochloric, sulfuric, or phosphoric acids because of the low pKa (estimated to be -0.7). The authors made some attempts to make amorphous forms but decided to look at cocrystals because of the risk of crystallization of the amorphous solid (low Tg). The authors screened for cocrystals with 26 different carboxylic acid-based coformers using the binary melt method on a hot-stage microscope. Five different new solid phases (cocrystals) were obtained and analyzed by spectroscopy. The authors selected the glutaric acid cocrystal2 for further evaluation because of the relatively high melting point of the cocrystal and the expected high water solubility of the cocrystal because of the high water solubility of the coformer. A solvent-based crystallization was developed to scale-up the process to make gram quantities of cocrystal.

Single-crystal X-ray data showed that one carboxylic group of the coformer hydrogen bonds to the amide functionality in an eight-membered ring motif, and the second acid group forms an interaction with a pyrimidine acceptor site. DSC thermal scans revealed that the cocrystal2 melts at 142°C, whereas compound 1 melts at 206°C. The cocrystal2 showed a significantly higher dissolution rate in water compared to compound 1. The intrinsic dissolution of the cocrystal was 18 times greater than compound 1.

The cocrystal2 was found to be nonhygroscopic as it sorbed less than 0.08% even at 95% RH. Both chemical and physical stability was good after 2 months of storage at 40°C/75% RH and 60°C. Though the cocrystal2 did convert to compound 1 in 37°C liquid water after 24 hours, the cocrystal2 did not convert to compound 1 during the 90 minutes required for the dissolution test or after 6 hours of exposure to 100% water vapor at RT. A dog PK study showed a significant increase in plasma concentration values after oral dosing of 2 relative to 1 at doses of 5 and 50 mg/kg. In summary, the authors reported the viability of the cocrystal as a crystalline solid form of a poorly soluble drug that was not amenable to salt formation.

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Metabolic and Degenerative Disorders of Childhood
Lucinda Carr, in Practical Guide to Neurogenetics, 2009

 

 

Glutaric Aciduria Type 1 (OMIM 231670)
The metabolism of lysine, hydroxylsine, and tryptophan is disrupted secondary to deficiency in glutaryl-CoA dehydrogenase, a mitochondrial enzyme. This results in the accumulation of glutaric acid and 3-hydroxyglutaric acid.

 

 

Clinical Features
Macrocephaly is an important early sign but clinical presentation is usually with progressive dystonia or choreoathetosis. Both acute and chronic presentations are seen. Early encephalitic crises are seen in around 70% of cases but become infrequent after 3 years of age. More chronic forms may be mistaken for cerebral palsy.

 

 

Investigation
Neuroimaging is characteristic, with frontotemporal atrophy and often subdural effusions or hematomas. This may lead to initial suspicion of child abuse. There is excessive glutaric and 3-hydroxyglutaric acid in the urine. Plasma free carnitine is reduced and glutaryl carnitine is elevated. Reduced enzyme activity is demonstrated in fibroblasts.

 

 

Neuropathology and Genetics
Striatal degeneration and spongiform white matter is often seen at autopsy.

 

The disorder is autosomal recessive and results from multiple mutations of the glutaryl-CoA dehydrogenase gene, located at 19p13.2, for which there is poor genotype-phenotype correlation.

 

Treatment
Treatment is with dietary restriction of lysine and tryptophan, supplemented with L-carnitine and riboflavin. Whilst this fails to improve existing neurological deficits, if started before any encephalitic crises subsequent deterioration may be averted. Supportive therapy should be given during intercurrent infections.

 

 

URINE ORGANIC ACIDS
Urine organic acid analysis detects a wide range of compounds. It is an excellent diagnostic test for the organic acidemias involving propionic, methylmalonic, and isovaleric acids. It also detects glutaric acid, which is a progressive neurotoxic defect in biomolecule conversion. The fatty acid oxidation defects also result in abnormal compounds in the urine. The presence of succinylacetone is a hallmark of tyrosinemia; similarly, the presence of isoleucine metabolites is a hallmark of maple syrup urine disease. Lactic acid and ketones are also detectable on organic acid analysis but are not always well correlated with plasma levels.

 

 

Accession Number T3D4359
Identification
Common Name Glutaric acid
Class Small Molecule
Description Glutaric acid is a simple five-carbon linear dicarboxylic acid. Glutaric acid is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Glutaric acid may cause irritation to the skin and eyes. When present in sufficiently high levels, glutaric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of glutaric acid are associated with at least three inborn errors of metabolism, including glutaric aciduria type I, malonyl-CoA decarboxylase deficiency, and glutaric aciduria type III. Glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH). Excessive levels of their intermediate breakdown products (e.g. glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid, glutaconic acid) can accumulate and cause damage to the brain (and also other organs). Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Macrocephaly is amongst the earliest signs of GA1. GA1 also causes secondary carnitine deficiency because glutaric acid, like other organic acids, is detoxified by carnitine. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to h ...Read more

 

 

Mechanism of Toxicity Accumulation of glutaric acid in the body has been shown to be toxic. The accumulation of glutaric acid ranging from slightly or intermittently elevated urinary glutaric acid to gross organic aciduria occurs in glutaric aciduria. Glutaric aciduria type 1 is an autosomal-recessive disorder resulting from a deficiency of mitochondrial glutaryl-CoA dehydrogenase which is involved in the metabolism of lysine, hydroxylysine, and tryptophan.
Metabolism Not Available
Toxicity Values Not Available
Lethal Dose Not Available
Carcinogenicity (IARC Classification) No indication of carcinogenicity to humans (not listed by IARC).
Uses/Sources This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation.
Minimum Risk Level Not Available
Health Effects Chronically high levels of glutaric acid are associated with at least 3 inborn errors of metabolism including: Glutaric Aciduria Type I and Glutaric Aciduria Type III.

 

 

Description belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.
Baric I, Wagner L, Feyh P, Liesert M, Buckel W, Hoffmann GF: Sensitivity and specificity of free and total glutaric acid and 3-hydroxyglutaric acid measurements by stable-isotope dilution assays for the diagnosis of glutaric aciduria type I. J Inherit Metab Dis. 1999 Dec;22(8):867-81. Pubmed: 10604139 
Bishop FS, Liu JK, McCall TD, Brockmeyer DL: Glutaric aciduria type 1 presenting as bilateral subdural hematomas mimicking nonaccidental trauma. Case report and review of the literature. J Neurosurg. 2007 Mar;106(3 Suppl):222-6. Pubmed: 17465389 
Goodman SI, Gallegos DA, Pullin CJ, Halpern B, Truscott RJ, Wise G, Wilcken B, Ryan ED, Whelen DT: Antenatal diagnosis of glutaric acidemia. Am J Hum Genet. 1980 Sep;32(5):695-9. Pubmed: 6893520 
Goodman SI, Stein DE, Schlesinger S, Christensen E, Schwartz M, Greenberg CR, Elpeleg ON: Glutaryl-CoA dehydrogenase mutations in glutaric acidemia (type I): review and report of thirty novel mutations. Hum Mutat. 1998;12(3):141-4. Pubmed: 9711871 
Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. Pubmed: 8087979 
Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. Pubmed: 8412012 
Hoffmann GF, Trefz FK, Barth PG, Bohles HJ, Biggemann B, Bremer HJ, Christensen E, Frosch M, Hanefeld F, Hunneman DH, et al.: Glutaryl-coenzyme A dehydrogenase deficiency: a distinct encephalopathy. Pediatrics. 1991 Dec;88(6):1194-203. Pubmed: 1956737 
Jakobs C, Sweetman L, Wadman SK, Duran M, Saudubray JM, Nyhan WL: Prenatal diagnosis of glutaric aciduria type II by direct chemical analysis of dicarboxylic acids in amniotic fluid. Eur J Pediatr. 1984 Jan;141(3):153-7. Pubmed: 6698061 
Muller E, Kolker S: Reduction of lysine intake while avoiding malnutrition--major goals and major problems in dietary treatment of glutaryl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 2004;27(6):903-10. Pubmed: 15505398 
Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. Pubmed: 2026685 
Singh I: Biochemistry of peroxisomes in health and disease. Mol Cell Biochem. 1997 Feb;167(1-2):1-29. Pubmed: 9059978 
Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. Pubmed: 19212411 
van der Werf, M. J., Overkamp, K. M., Muilwijk, B., Coulier, L., Hankemeier, T. (2007). "Microbial metabolomics: toward a platform with full metabolome coverage." Anal Biochem 370:17-25. Pubmed: 17765195 
Whelan DT, Hill R, Ryan ED, Spate M: L-Glutaric acidemia: investigation of a patient and his family. Pediatrics. 1979 Jan;63(1):88-93. Pubmed: 440804 
Yurtsever D. (2007). Fatty acid methyl ester profiling of Enterococcus and Esherichia coli for microbial source tracking. M.sc. Thesis. Villanova University: U.S.A
Synthesis Reference: Petrova, E. G.; Kasumova, N. M.; Kagramanova, N. A.; Dadasheva, A. D. Mechanism of glutaric acid synthesis. Issledovaniya v Oblasti Kinetiki, Modelirovaniya i Optimizatsii Khimicheskikh Protsessov (1974), 2 277-89.
Material Safety Data Sheet (MSDS) Download (PDF)
Links

 

 

Clinical Information 
Acylcarnitine analysis is included in newborn screening blood testing and is utilized for detection of several inborn errors of metabolism, including fatty acid oxidation disorders (FAOD) and organic acidemias (OA). A limitation of this analytic method is its inability to differentiate between several isomers. Additional testing of 2-hydroxy glutaric acid (2OH-GA), 3-hydroxy glutaric acid (3OH-GA), glutaric acid (GA), methylsuccinic acid (MSA), and ethylmalonic acid (EMA) by LC-MS/MS allows better differentiation among C4-acylcarnitine and glutarylcarnitine/C10-OH isomers.

 

C4-acylcarnitine represents both butyrylcarnitine and isobutyrylcarnitine and is elevated in short-chain acyl Co-A dehydrogenase (SCAD) deficiency, isobutyryl-CoA dehydrogenase (IBDH) deficiency, and ethylmalonic encephalopathy (EE). SCAD deficiency is a condition affecting fatty acid metabolism, with reported symptoms of hypoglycemia, lethargy, developmental delays, and failure to thrive. There is controversy on whether a biochemical diagnosis necessarily confers clinical symptoms. IBDH deficiency is characterized by cardiomyopathy, hypotonia, and developmental delays, although many individuals with IBDH deficiency are asymptomatic. EE is a rare progressive encephalopathy associated with hypotonia, seizures, and abnormal movements.

Individuals with SCAD deficiency demonstrate elevated plasma EMA and MSA levels and individuals with EE show only elevations in EMA, while individuals with IBDH deficiency do not typically have elevations in either EMA or MSA.

Glutarylcarnitine (C5-DC) is elevated in glutaric acidemia type 1 (GA-1), but is not differentiated from C10-OH acylcarnitine. GA-1, is caused by a deficiency of glutaryl-CoA dehydrogenase and is characterized by bilateral striatal brain injury leading to dystonia, often a result of acute neurologic crises triggered by illness. Individuals with GA-1 typically show elevations of glutaric acid and 3OH-GA, even in those considered to be "low excretors."

Glutaric acidemia (GA-2), also known as multiple acyl-CoA dehydrogenase deficiency (MADD), is caused by defects in either the electron transfer flavoprotein (ETF) or ETF-ubiquinone oxidoreductase. This disease can be severe and is often fatal in the first weeks of life, with typical symptoms of hypoglycemia, muscle weakness, metabolic acidosis, dysmorphic features, cardiac defects or arrhythmias, renal cysts, and fatty infiltration of the liver. GA-2 can have a milder presentation, also known as ethylmalonic-adipic aciduria, with Reye-like illnesses in childhood and muscle weakness in childhood and adulthood. In addition to elevations in glutaric acid, individuals with GA-2 can also show increased EMA, MSA, and 2OH-GA.

The American College of Medical Genetics (ACMG) newborn screening work group published diagnostic algorithms for the follow-up of infants who had a positive newborn screening result. For more information, see www.acmg.net.

 

Reference Values 
2-OH Glutaric acid: < or =25 nmol/mL

 

3-OH Glutaric acid: < or =1.5 nmol/mL

Glutaric acid: < or =1.5 nmol/mL

Methylsuccinic acid: < or =0.45 nmol/mL

Ethylmalonic acid: < or =3.5 nmol/mL

 

Interpretation 
Elevations of ethylmalonic acid (EMA) and methylsuccinic acid (MSA) are consistent with a diagnosis of short-chain acyl Co-A dehydrogenase (SCAD) deficiency.

 

Elevation of EMA is consistent with a diagnosis of ethylmalonic encephalopathy.

Normal levels of EMA in the context of elevated C4 is consistent with a diagnosis of isobutyryl-CoA dehydrogenase (IBDH) deficiency.

Elevation of glutaric acid (GA) and 3-hydroxy glutaric acid (3OH-GA) are consistent with a diagnosis of glutaric acidemia type 1 (GA-1).

Elevation of GA, 2-hydroxy glutaric acid (2OH-GA), 3OH-GA, EMA, and MSA are consistent with a diagnosis of glutaric acidemia (GA-2).

 

Cautions 
No significant cautionary statements

 

 

Clinical Reference 
1. Rinaldo P, Cowan TM, Matern D: Acylcarnitine profile analysis. 2008:10(2):151-156

 

2. Kolker S, Christensen E, Leonar JV, et al: Diagnosis and management of glutaric aciduria type I-revised recommendations. J Inherit Metab Dis 2011;34:677-694

3. Frerman FE, Goodman SI: Chapter 103: Defects of electron transfer flavoprotein and electron transfer flavoprotein-ubiquinone oxidoreductase: Glutaric Acidemia Type II. In Scriver's Online Metabolic and Molecular Bases of Inherited Disease. Edited by CR Scriver, AL Beaudet, D Valle, et al. Accessed 8/17/17. Available at www.ommbid.com/

 

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CHEBI:17859 - glutaric acid
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ChEBI Name glutaric acid
ChEBI ID CHEBI:17859
Definition An α,ω-dicarboxylic acid that is a linear five-carbon dicarboxylic acid.
Stars This entity has been manually annotated by the ChEBI Team.
Secondary ChEBI IDs CHEBI:43097, CHEBI:5434, CHEBI:24330
Supplier Information ChemicalBook:CB7852828, eMolecules:515060, MolPort-001-759-254, ZINC000000388706
Download Molfile XML SDF
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Wikipedia License 
Glutaric acid is the organic compound with the formula C3H6(COOH)2 . Although the related "linear" dicarboxylic acids adipic and succinic acids are water-soluble only to a few percent at room temperature, the water-solubility of glutaric acid is over 50% (w/w).
Read full article at Wikipedia

 

 

Formula C5H8O4
Net Charge 0
Average Mass 132.11462
Monoisotopic Mass 132.04226
InChI InChI=1S/C5H8O4/c6-4(7)2-1-3-5(8)9/h1-3H2,(H,6,7)(H,8,9)
InChIKey JFCQEDHGNNZCLN-UHFFFAOYSA-N
SMILES OC(=O)CCCC(O)=O
Metabolite of Species Details
Daphnia magna (NCBI:txid35525) See: Mixtures of similarly acting compounds in Daphnia magna: From gene to metabolite and beyondTine Vandenbrouck, Oliver A.H. Jones, Nathalie Dom, Julian L. Griffin, Wim De CoenEnvironment International 36 (2010) 254-268
Homo sapiens (NCBI:txid9606) See: PubMed
Roles Classification 
Chemical Role(s): Bronsted acid
A molecular entity capable of donating a hydron to an acceptor (Bronsted base).
(via oxoacid )
Biological Role(s): human metabolite
Any mammalian metabolite produced during a metabolic reaction in humans (Homo sapiens).
Daphnia magna metabolite
A Daphnia metabolite produced by the species Daphnia magna.
View more via ChEBI Ontology
ChEBI Ontology 
Outgoing glutaric acid (CHEBI:17859) has role Daphnia magna metabolite (CHEBI:83056) 
glutaric acid (CHEBI:17859) has role human metabolite (CHEBI:77746) 
glutaric acid (CHEBI:17859) is a α,ω-dicarboxylic acid (CHEBI:28383) 
glutaric acid (CHEBI:17859) is conjugate acid of glutarate (CHEBI:24329) 
glutaric acid (CHEBI:17859) is conjugate acid of glutarate(1-) (CHEBI:35907) 
Incoming (2S)-2-(3-{2-bromo-5-[(2,4-diaminopyrimidin-1-ium-5-yl)methyl]-3-methoxyphenoxy}propyl)pentanedioate (CHEBI:47236) has functional parent glutaric acid (CHEBI:17859)
(R)-1,2-di-O-dodecanylglycero-3-glutaric acid 6ʼ-methylresorufin ester (CHEBI:131618) has functional parent glutaric acid (CHEBI:17859)
(S)-1,2-di-O-dodecanylglycero-3-glutaric acid 6ʼ-methylresorufin ester (CHEBI:100462) has functional parent glutaric acid (CHEBI:17859)
1-(2-{4-[(4-carboxybutanoyl)amino]phenyl}ethyl)-1-methylpiperidinium (CHEBI:42893) has functional parent glutaric acid (CHEBI:17859)
1-hexadecyl-2-glutaryl-sn-glycero-3-phosphocholine (CHEBI:79276) has functional parent glutaric acid (CHEBI:17859)
2-formylglutaric acid (CHEBI:49075) has functional parent glutaric acid (CHEBI:17859)
2-hydroxyglutaric acid (CHEBI:17084) has functional parent glutaric acid (CHEBI:17859)
2-hydroxymethylglutaric acid (CHEBI:49077) has functional parent glutaric acid (CHEBI:17859)
2-methylglutaric acid (CHEBI:68567) has functional parent glutaric acid (CHEBI:17859)
2-methylideneglutaric acid (CHEBI:17207) has functional parent glutaric acid (CHEBI:17859)
2-oxoglutaric acid (CHEBI:30915) has functional parent glutaric acid (CHEBI:17859)
3,3-dimethylglutaric acid (CHEBI:68502) has functional parent glutaric acid (CHEBI:17859)
3-hydroxy-3-methylglutaric acid (CHEBI:16831) has functional parent glutaric acid (CHEBI:17859)
3-methylglutaric acid (CHEBI:68566) has functional parent glutaric acid (CHEBI:17859)
4-(4-styrylphenylcarbamoyl)butyric acid (CHEBI:45573) has functional parent glutaric acid (CHEBI:17859)
4-hydroxy-2-oxoglutaric acid (CHEBI:30923) has functional parent glutaric acid (CHEBI:17859)
4-hydroxy-4-methyl-2-oxoglutaric acid (CHEBI:17801) has functional parent glutaric acid (CHEBI:17859)
5-({4-[2-(1-oxidopiperidin-1-yl)ethyl]phenyl}amino)-5-oxopentanoic acid (CHEBI:44598) has functional parent glutaric acid (CHEBI:17859)
5-{[4-({hydroxy[(4-nitrophenyl)amino]phosphoryl}methyl)phenyl]amino}-5-oxopentanoic acid (CHEBI:63478) has functional parent glutaric acid (CHEBI:17859)
8-benzyl-7-hydroxy-4,10-dioxo-1-phenyl-6-oxa-3,9-diaza-7-phosphatetradecan-14-oic acid 7-oxide (CHEBI:43081) has functional parent glutaric acid (CHEBI:17859)
N-formylisoglutamic acid (CHEBI:28904) has functional parent glutaric acid (CHEBI:17859)
O-glutaroyl-L-carnitine (CHEBI:82952) has functional parent glutaric acid (CHEBI:17859)
O-glutarylcarnitine (CHEBI:73040) has functional parent glutaric acid (CHEBI:17859)
p-nitrobenzyl glutaryl glycinic acid (CHEBI:44939) has functional parent glutaric acid (CHEBI:17859)
diethyl glutarate (CHEBI:87319) has functional parent glutaric acid (CHEBI:17859)
glutaramic acid (CHEBI:24326) has functional parent glutaric acid (CHEBI:17859)
glutaryl-CoAs (CHEBI:24333) has functional parent glutaric acid (CHEBI:17859)
isoglutamic acid (CHEBI:28791) has functional parent glutaric acid (CHEBI:17859)
monomethyl glutaric acid (CHEBI:86396) has functional parent glutaric acid (CHEBI:17859)
glutarate (CHEBI:24329) is conjugate base of glutaric acid (CHEBI:17859)
glutarate(1-) (CHEBI:35907) is conjugate base of glutaric acid (CHEBI:17859)
IUPAC Name 
pentanedioic acid
Synonyms Sources
1,3-Propanedicarboxylic acid KEGG COMPOUND
1,5-pentanedioic acid NIST Chemistry WebBook Indicates when synonyms have been adapted from the specified source i.e. not exact copies.
Glutaric acid KEGG COMPOUND
GLUTARIC ACID PDBeChem
Glutarsäure Deutsch ChEBI
Pentanedioic acid KEGG COMPOUND
Manual Xrefs Databases
C00001184 KNApSAcK
C00489 KEGG COMPOUND
DB03553 DrugBank
Glutaric_acid Wikipedia
GUA PDBeChem
HMDB0000661 HMDB
LMFA01170046 LIPID MAPS
View more database links
Registry Numbers Types Sources
110-94-1 CAS Registry Number KEGG COMPOUND
110-94-1 CAS Registry Number NIST Chemistry WebBook
110-94-1 CAS Registry Number ChemIDplus
1209725 Reaxys Registry Number Reaxys
1209725 Beilstein Registry Number Beilstein
26809 Gmelin Registry Number Gmelin
Citations 
Tian F, Fu X, Gao J, Ying Y, Hou L, Liang Y, Ning Q, Luo X (2014)
Glutaric acid-mediated apoptosis in primary striatal neurons. 
BioMed research international 2014, 484731 [PubMed:24900967]
[show Abstract]
Pusti S, Das N, Nayek K, Biswas S (2014)
A treatable neurometabolic disorder: glutaric aciduria type 1. 
Case reports in pediatrics 2014, 256356 [PubMed:24587932]
[show Abstract]
Olivera-Bravo S, Isasi E, Fernández A, Rosillo JC, Jiménez M, Casanova G, Sarlabós MN, Barbeito L (2014)
White matter injury induced by perinatal exposure to glutaric acid. 
Neurotoxicity research 25, 381-391 [PubMed:24297153]
[show Abstract]
Last Modified 
09 August 2016
BioMed Research International
Volume 2014, Article ID 484731, 10 pages
http://dx.doi.org/10.1155/2014/484731
Research Article
Glutaric Acid-Mediated Apoptosis in Primary Striatal Neurons
Fengyan Tian,1,2 Xi Fu,1 Jinzhi Gao,1 Yanqin Ying,1 Ling Hou,1 Yan Liang,1 Qin Ning,3 and Xiaoping Luo1
1Department of Pediatrics, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, China
2Department of Pediatrics, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, China
3Laboratory of Infectious Immunology, Department of Infectious Disease, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, China

 

Received 12 February 2014; Revised 20 April 2014; Accepted 21 April 2014; Published 12 May 2014

Academic Editor: Janet K. Kern

Copyright © 2014 Fengyan Tian et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

 

Abstract
Glutaric acid (GA) has been implicated in the mechanism of neurodegeneration in glutaric aciduria type I. In the present study, the potential cytotoxic effects of GA (0.1~50 mM for 24~96 h) were examined in cultured primary rat striatal neurons. Results showed increase in the number of cells labeled by annexin-V or with apoptotic features shown by Hoechst/PI staining and transmission electron microscopy (TEM) and upregulation of the expression of mRNA as well as the active protein fragments caspase 3, suggesting involvement of the caspase 3-dependent apoptotic pathway in GA-induced striatal neuronal death. This effect was in part suppressed by the N-methyl-D-aspartate (NMDA) receptor antagonist MK-801 but not the α-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) antagonist 6-cyano-7-nitroquinoxalone-2,3-dione (CNQX). Thus, GA may trigger neuronal damage partially through apoptotic pathway and via activation of NMDA receptors in cultured primary striatal neurons.

 

 

1. Introduction
Glutaric aciduria type I (GA I) is an autosomal recessive organic aciduria caused by mutations in the gene encoding glutaryl-CoA dehydrogenase (GCDH). Patients are at risk of developing an irreversible dystonia and/or dyskinesia movement disorders as a sequence of selective destruction of striatal neurons following acute encephalopathic crises triggered by intercurrent catabolic situations such as infectious diseases, fever, vomiting, diarrhea, or immunization. The acute episodes of encephalopathy usually occurred within the first 3 years of life [1]. The main pathomorphological findings are frontotemporal atrophy, basal ganglia lesions, white matter disease, and postsynaptic vacuolization of neurons [2]. However, the clinical phenotype has no relationship to the biochemical phenotype or the genotype [1, 3].

 

 

Figure 7: Rates of apoptosis in cells treated with 0, 30, and 50 mM GA for 24 h, in the presence or absence of 10 μM MK-801 or CNQX, assayed by flow cytometry. and , compared to normal control cells; and , compared to cells treated with 30 mM GA and no antagonist. and , compared to cells treated with 50 mM GA and no antagonist.
3.5. Electron Microscopic Study
Striatal neurons exposed to 30 mM GA for 24 h showed characteristic condensation of nuclear chromatin (Figure 8(b)). A normal neuron was shown in Figure 8(a) for comparison.

 

 

fig8
Figure 8: Apoptosis examined by TEM. (a) Striatal neuron with normal ultrastructure. (b) Typical apoptotic cell (intermediate-late stage) with shrunken nucleus and condensed chromatin.
3.6. Effect of Glutaric Acid on Caspase 3 Transcript and Protein Levels
Quantitative RT-PCR was performed to monitor mRNA expression of the apoptotic executioner caspase 3 (Figure 9(a)). The comparative method was used to analyse relative expression levels. Caspase 3 mRNA expression at 6 hours after treatment with 1, 10, 25, and 50 mM GA was upregulated about 1.40-fold, 1.67-fold, and 1.95-fold, respectively, compared to control. Thus GA might induce apoptosis via caspase 3 activation.

 

 

4. Discussion
Glutaric aciduria type I is an autosomal recessive disorder characterized by high levels of GA, 3-hydroxyglutaric acid (3-OHGA), glutaconic acid, and glutaryl-CoA in body fluids as well as degenerative changes in the striatal and frontotemporal cortical neurons. A deficiency of cerebral GCDH activity is attributed to the development of neurological damage in GA I patients. However, the comprehension of the degeneration mechanism in the basal ganglia still remains partial.

 

 

Synonyms of Glutaric Aciduria Type I
GA1
glutaric acidemia type I
glutaric aciduria type I
glutaryl-CoA dehydrogenase deficiency
General Discussion
Glutaric aciduria type I (GA1) is a rare hereditary metabolic disorder caused by a deficiency of the mitochondrial enzyme glutaryl-CoA dehydrogenase (GCDH). It is in the group of disorders known as cerebral organic acidemias. Individuals with this condition have deficiency or absence of GCDH enzyme that is involved in the lysine metabolism. GCDH deficiency results in increased concentrations of potentially neurotoxic metabolites, glutaric acid (GA), 3-hydroxy glutaric acid (3-OH-GA) and glutaconic acid within body tissues, especially within the brain. Two arbitrary biochemical subtypes have been defined, high (HE) and low excretors (LE), depending on the amount of GA in the urine. Newborns may show unspecific clinical signs like enlarged head circumference (macrocephaly) or decreased muscle tone (hypotonia). Without treatment, most affected children develop an acute encephalopathic crisis following febrile illness episodes or other catabolic conditions resulting in bilateral striatal injury and consequently, dystonic movement disorder. Cognitive outcome has not been systematically studied, but severe cognitive dysfunction is rarely seen. Sometimes babies with GA1 have been mistaken to have been abused because they present with subdural and/or retinal hemorrhages. GA1 is included in the newborn screening panel in a growing number of countries which is essential for early intervention. Importantly, patients with the low excreting phenotype may be missed by newborn screening.

 

Glutaric aciduria type II is a totally different disease and belongs to the group of fatty acid oxidation disorders. These are metabolic disorders characterized by a lack of the enzymes are needed to break down fats, resulting in delayed mental and physical development. Two forms of this disorder occur during different stages of life.

1) Glutaric aciduria IIA (GA IIA) is the neonatal form of glutaricaciduria II. This form of glutaric aciduria II is a very rare, X-linked hereditary disorder characterized by large amounts of glutaric and other acids in blood and urine. The disorder is caused by dysfunction of the electron-transferring flavoprotein in the mitochondria.

2) Glutarica aciduria IIB (GA IIB; ethylmalonic adipicaciduria) is the adult form of glutaricaciduria II. This milder form of the disorder is inherited in an autosomal recessive pattern. Acidity of the body tissues (metabolic acidosis), and a low blood sugar level (hypoglycemia) without an elevated level of ketones in body tissues (ketosis), occur during adulthood. Large amounts of glutaric acid in the blood and urine are caused by a deficiency of the enzyme multiple acyl-CoA dehydrogenase. (For more information on this disorder, choose "glutaric aciduria II" as your search term in the Rare Disease Database.)

Glutaric aciduria III is an autosomal recessive genetic condition characterized by accumulation or excretion of glutaric acid and caused by mutations in the C7ORF10 gene. Symptoms vary and some individuals show no symptoms

Goodman SI, Frerman FE. Organic acidemias due to defects in lysine oxidation: 2-ketoadipic acidemia and glutaric acidemia. In: Scriver CR, Beaudet AL, Sly WS, et al. Eds. The Metabolic Molecular Basis of Inherited Disease. 7th ed. McGraw-Hill Companies. New York, NY; 1995:1451-60.

JOURNAL ARTICLES

Peters V, Morath M, Mack M, et al. Formation of 3-hydroxyglutaric acid in glutaric aciduria type I: in vitro participation of medium chain acyl-CoA dehydrogenase. JIMD Rep. 2019;47(1):30-34. Published 2019 Mar 26. doi:10.1002/jmd2.12026

Boy N, Mengler K, Thimm E, et al Newborn screening: A disease-changing intervention for glutaric aciduria type 1. Ann Neurol. 2018 May;83(5):970-979. doi: 10.1002/ana.25233. Epub 2018 Apr 30.

Boy N, Mühlhausen C, Maier EM, et al. Proposed recommendations for diagnosing and managing individuals with glutaric aciduria type I: second revision. J Inherit Metab Dis. 2017 Jan;40(1):75-101. doi: 10.1007/s10545-016-9999-9. Epub 2016 Nov 16. Review. PubMed PMID: 27853989.select

Mosaeilhy A, Mohamed MM, C GPD, et al. Genotype-phenotype correlation in 18 Egyptian patients with glutaric acidemia type I. Metab Brain Dis. 2017 Oct;32(5):1417-1426. doi: 10.1007/s11011-017-0006-4. Epub 2017 Apr 7. PubMed PMID: 28389991.

Hedlund GL, Longo N, Pasquali M. Glutaric acidemia type 1. Am J Med Genet C Semin Med Genet. 2006 May 15;142C(2):86-94. doi: 10.1002/ajmg.c.30088. Review. PubMed PMID: 16602100; PubMed Central PMCID: PMC2556991.

Bähr O, Mader I, Zschocke J, Dichgans J, Schulz JB. Adult onset glutaric aciduria type I presenting with a leukoencephalopathy. Neurology. 2002 Dec 10;59(11):1802-4. doi: 10.1212/01.wnl.0000036616.11962.3c. PubMed PMID: 12473778.

Kölker S, Ramaekers VT, Zschocke J, Hoffmann GF. Acute encephalopathy despite early therapy in a patient with homozygosity for E365K in the glutaryl-coenzyme A dehydrogenase gene. J Pediatr. 2001 Feb;138(2):277-9. doi: 10.1067/mpd.2001.110303. PubMed PMID: 11174631.select

Busquets C, Coll MJ, Merinero B, et al. Prenatal molecular diagnosis of glutaric aciduria type I by direct mutation analysis. Prenat Diagn. 2000 Sep;20(9):761-4. PubMed PMID: 11015709.

Kafil-Hussain NA, Monavari A, Bowell R, Thornton P, Naughten E, O'Keefe M. Ocular findings in glutaric aciduria type 1. J Pediatr Ophthalmol Strabismus. 2000 Sep-Oct;37(5):289-93. PubMed PMID: 11020111.

Zafeiriou DI, Zschocke J, Augoustidou-Savvopoulou P, et al. Atypical and variable clinical presentation of glutaric aciduria type I. Neuropediatrics. 2000 Dec;31(6):303-6. doi: 10.1055/s-2000-12943. PubMed PMID: 11508549.

Baric I, Wagner L, Feyh P, Liesert M, Buckel W, Hoffmann GF. Sensitivity and specificity of free and total glutaric acid and 3-hydroxyglutaric acid measurements by stable-isotope dilution assays for the diagnosis of glutaric aciduria type I. J Inherit Metab Dis.1999 Dec;22(8):867-81. PubMed PMID: 10604139.

Hoffmann GF, Zschocke J. Glutaric aciduria type I: from clinical, biochemical and molecular diversity to successful therapy. J Inherit Metab Dis. 1999 Jun;22(4):381-91. Review. PubMed PMID: 10407775

 

Naylor EW, Chace DH. Automated tandem mass spectrometry for mass newborn screening for disorders in fatty acid, organic acid, and amino acid metabolism. J Child Neurol. 1999 Nov;14 Suppl 1:S4-8. doi: 10.1177/0883073899014001021. PubMed PMID: 10593560.
Glutaric acid
Class Small Molecule
Description Glutaric acid is a simple five-carbon linear dicarboxylic acid. Glutaric acid is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Glutaric acid may cause irritation to the skin and eyes. When present in sufficiently high levels, glutaric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of glutaric acid are associated with at least three inborn errors of metabolism, including glutaric aciduria type I, malonyl-CoA decarboxylase deficiency, and glutaric aciduria type III. Glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH). Excessive levels of their intermediate breakdown products (e.g. glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid, glutaconic acid) can accumulate and cause damage to the brain (and also other organs). Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Macrocephaly is amongst the earliest signs of GA1. GA1 also causes secondary carnitine deficiency because glutaric acid, like other organic acids, is detoxified by carnitine. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to h ...Read more

 

 

DRUG INTERACTION
Acetazolamide The excretion of Glutaric Acid can be decreased when combined with Acetazolamide.
Acetylsalicylic acid The excretion of Glutaric Acid can be decreased when combined with Acetylsalicylic acid.
Acyclovir The excretion of Glutaric Acid can be decreased when combined with Acyclovir.
Adefovir dipivoxil The excretion of Glutaric Acid can be decreased when combined with Adefovir dipivoxil.
Allopurinol The excretion of Allopurinol can be decreased when combined with Glutaric Acid.
Alprostadil The excretion of Alprostadil can be decreased when combined with Glutaric Acid.
Aminohippuric acid The excretion of Glutaric Acid can be decreased when combined with Aminohippuric acid.
Aminophenazone The excretion of Glutaric Acid can be decreased when combined with Aminophenazone.
Amoxicillin The excretion of Glutaric Acid can be decreased when combined with Amoxicillin.
Antipyrine The excretion of Glutaric Acid can be decreased when combined with Antipyrine.

 

Guiseppe Gigliotti, Jean-Michel Roul, "Process for the preparation of 3-methyl-3-hydroxy-glutaric acid." U.S. Patent US4467108, issued June, 1980.

Diagnosis of glutaric acidemia type 1

Aids in diagnosis of glutaric acidemia type 2

 

Genetics Test Information 
Second-tier newborn screening for follow-up of C4-acylcarnitine and glutarylcarnitine (C5DC) elevations.

 

Differentiating diagnoses of short-chain Co-A dehydrogenase (SCAD) deficiency, isobutyryl-CoA dehydrogenase (IBDH) deficiency, and ethylmalonic encephalopathy.

Differentiating diagnoses of glutaric acidemia type I (GA-1) and glutaric acidemia type II (GA-2)

 

Clinical Information 
Acylcarnitine analysis is included in newborn screening blood testing and is utilized for detection of several inborn errors of metabolism, including fatty acid oxidation disorders (FAOD) and organic acidemias (OA). A limitation of this analytic method is its inability to differentiate between several isomers. Additional testing of 2-hydroxy glutaric acid (2OH-GA), 3-hydroxy glutaric acid (3OH-GA), glutaric acid (GA), methylsuccinic acid (MSA), and ethylmalonic acid (EMA) by LC-MS/MS allows better differentiation among C4-acylcarnitine and glutarylcarnitine/C10-OH isomers.

 

C4-acylcarnitine represents both butyrylcarnitine and isobutyrylcarnitine and is elevated in short-chain acyl Co-A dehydrogenase (SCAD) deficiency, isobutyryl-CoA dehydrogenase (IBDH) deficiency, and ethylmalonic encephalopathy (EE). SCAD deficiency is a condition affecting fatty acid metabolism, with reported symptoms of hypoglycemia, lethargy, developmental delays, and failure to thrive. There is controversy on whether a biochemical diagnosis necessarily confers clinical symptoms. IBDH deficiency is characterized by cardiomyopathy, hypotonia, and developmental delays, although many individuals with IBDH deficiency are asymptomatic. EE is a rare progressive encephalopathy associated with hypotonia, seizures, and abnormal movements.

Individuals with SCAD deficiency demonstrate elevated plasma EMA and MSA levels and individuals with EE show only elevations in EMA, while individuals with IBDH deficiency do not typically have elevations in either EMA or MSA.

Glutarylcarnitine (C5-DC) is elevated in glutaric acidemia type 1 (GA-1), but is not differentiated from C10-OH acylcarnitine. GA-1, is caused by a deficiency of glutaryl-CoA dehydrogenase and is characterized by bilateral striatal brain injury leading to dystonia, often a result of acute neurologic crises triggered by illness. Individuals with GA-1 typically show elevations of glutaric acid and 3OH-GA, even in those considered to be "low excretors."

Glutaric acidemia (GA-2), also known as multiple acyl-CoA dehydrogenase deficiency (MADD), is caused by defects in either the electron transfer flavoprotein (ETF) or ETF-ubiquinone oxidoreductase. This disease can be severe and is often fatal in the first weeks of life, with typical symptoms of hypoglycemia, muscle weakness, metabolic acidosis, dysmorphic features, cardiac defects or arrhythmias, renal cysts, and fatty infiltration of the liver. GA-2 can have a milder presentation, also known as ethylmalonic-adipic aciduria, with Reye-like illnesses in childhood and muscle weakness in childhood and adulthood. In addition to elevations in glutaric acid, individuals with GA-2 can also show increased EMA, MSA, and 2OH-GA.

The American College of Medical Genetics (ACMG) newborn screening work group published diagnostic algorithms for the follow-up of infants who had a positive newborn screening result. For more information, see www.acmg.net.

 

Reference Values 
2-OH Glutaric acid: < or =25 nmol/mL

 

3-OH Glutaric acid: < or =1.5 nmol/mL

Glutaric acid: < or =1.5 nmol/mL

Methylsuccinic acid: < or =0.45 nmol/mL

Ethylmalonic acid: < or =3.5 nmol/mL

 

Interpretation 
Elevations of ethylmalonic acid (EMA) and methylsuccinic acid (MSA) are consistent with a diagnosis of short-chain acyl Co-A dehydrogenase (SCAD) deficiency.

 

Elevation of EMA is consistent with a diagnosis of ethylmalonic encephalopathy.

Normal levels of EMA in the context of elevated C4 is consistent with a diagnosis of isobutyryl-CoA dehydrogenase (IBDH) deficiency.

Elevation of glutaric acid (GA) and 3-hydroxy glutaric acid (3OH-GA) are consistent with a diagnosis of glutaric acidemia type 1 (GA-1).

Elevation of GA, 2-hydroxy glutaric acid (2OH-GA), 3OH-GA, EMA, and MSA are consistent with a diagnosis of glutaric acidemia (GA-2).

 

Cautions 
No significant cautionary statements

 

 

Clinical Reference 
1. Rinaldo P, Cowan TM, Matern D: Acylcarnitine profile analysis. 2008:10(2):151-156

 

2. Kolker S, Christensen E, Leonar JV, et al: Diagnosis and management of glutaric aciduria type I-revised recommendations. J Inherit Metab Dis 2011;34:677-694

3. Frerman FE, Goodman SI: Chapter 103: Defects of electron transfer flavoprotein and electron transfer flavoprotein-ubiquinone oxidoreductase: Glutaric Acidemia Type II. In Scriver's Online Metabolic and Molecular Bases of Inherited Disease. Edited by CR Scriver, AL Beaudet, D Valle, et al. Accessed 8/17/17. Available at www.ommbid.com/

 

Special Instructions 
Biochemical Genetics Patient Information
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Glutaric Aciduria Type I
NORD gratefully acknowledges Bridget McClain and Ashby Martin, NORD Editorial Interns from the University of Notre Dame; Barb Calhoun, MSN, RN, NP, Nurse Practitioner and Outreach Coordinator, Boler-Parseghian Center for Rare and Neglected Diseases at the University of Notre Dame; and Nikolas Boy, MD, Metabolic Pediatrician, Centre for Child and Adolescent Medicine, Division of Child Neurology and Metabolic Medicine, Heidelberg University Hospital, for assistance in the preparation of this report.

 

 

Synonyms of Glutaric Aciduria Type I
GA1
glutaric acidemia type I
glutaric aciduria type I
glutaryl-CoA dehydrogenase deficiency
General Discussion
Glutaric aciduria type I (GA1) is a rare hereditary metabolic disorder caused by a deficiency of the mitochondrial enzyme glutaryl-CoA dehydrogenase (GCDH). It is in the group of disorders known as cerebral organic acidemias. Individuals with this condition have deficiency or absence of GCDH enzyme that is involved in the lysine metabolism. GCDH deficiency results in increased concentrations of potentially neurotoxic metabolites, glutaric acid (GA), 3-hydroxy glutaric acid (3-OH-GA) and glutaconic acid within body tissues, especially within the brain. Two arbitrary biochemical subtypes have been defined, high (HE) and low excretors (LE), depending on the amount of GA in the urine. Newborns may show unspecific clinical signs like enlarged head circumference (macrocephaly) or decreased muscle tone (hypotonia). Without treatment, most affected children develop an acute encephalopathic crisis following febrile illness episodes or other catabolic conditions resulting in bilateral striatal injury and consequently, dystonic movement disorder. Cognitive outcome has not been systematically studied, but severe cognitive dysfunction is rarely seen. Sometimes babies with GA1 have been mistaken to have been abused because they present with subdural and/or retinal hemorrhages. GA1 is included in the newborn screening panel in a growing number of countries which is essential for early intervention. Importantly, patients with the low excreting phenotype may be missed by newborn screening.

 

 

Signs & Symptoms
Babies with GA1 are born healthy and may only present with unspecific signs like macrocephaly at birth. Macrocephaly is one of the earliest signs of GA1 so newborns with an enlarged head circumference should be evaluated for GA1. Especially during the age of 3 months until 3 years, most affected babies develop an acute encephalopathic crisis which is triggered by catabolic conditions such as febrile infections, febrile reactions to vaccinations or surgery. These crises result in striatal injury and a complex, mostly dystonic, irreversible and severe movement disorder which is associated with high morbidity and mortality. These babies can experience several symptoms resembling those of cerebral palsy, such as frequently assuming odd positions due to disordered muscle tone (dystonia), involuntary and ceaseless slow, sinuous, writhing (athetotic) or jerky (choreic) movements of the trunk and limbs. Controlling the movement of hands, arms, feet, legs, head, and neck may become very hard and muscle spasms may occur. Repeated stress on the body (such as infection and fever) can cause symptoms to worsen, but in some children, brain damage will occur without a triggering fever. Mild to moderate intellectual disabilities may also accompany these symptoms, but some studies have shown that the intellectual ability of a person with GA1, even if untreated, is not or only mildly affected.

 

In recent years, also other neurologic disease manifestations have been reported such as chronic kidney disease.

For 80-90% of people with GA1, motor symptom development is preventable, but this requires early diagnosis by newborn screening and treatment from birth on. Treatment consists of a low lysine diet and oral carnitine supplementation as well as intermittent emergency treatment during episodes that are likely to induce catabolism. If treatment is delayed or inadequate, motor symptoms begin to manifest acutely or insidiously during infancy or early childhood (before the age of 6) and are often highly variable. A minority of GA1 patients is diagnosed lately in adolescence or even adult age, following the diagnostic work-up of unspecific neurologic symptoms.

1) Glutaric aciduria IIA (GA IIA) is the neonatal form of glutaricaciduria II. This form of glutaric aciduria II is a very rare, X-linked hereditary disorder characterized by large amounts of glutaric and other acids in blood and urine. The disorder is caused by dysfunction of the electron-transferring flavoprotein in the mitochondria.

2) Glutarica aciduria IIB (GA IIB; ethylmalonic adipicaciduria) is the adult form of glutaricaciduria II. This milder form of the disorder is inherited in an autosomal recessive pattern. Acidity of the body tissues (metabolic acidosis), and a low blood sugar level (hypoglycemia) without an elevated level of ketones in body tissues (ketosis), occur during adulthood. Large amounts of glutaric acid in the blood and urine are caused by a deficiency of the enzyme multiple acyl-CoA dehydrogenase. (For more information on this disorder, choose "glutaric aciduria II" as your search term in the Rare Disease Database.)

Glutaric aciduria III is an autosomal recessive genetic condition characterized by accumulation or excretion of glutaric acid and caused by mutations in the C7ORF10 gene. Symptoms vary and some individuals show no symptoms

Goodman SI, Frerman FE. Organic acidemias due to defects in lysine oxidation: 2-ketoadipic acidemia and glutaric acidemia. In: Scriver CR, Beaudet AL, Sly WS, et al. Eds. The Metabolic Molecular Basis of Inherited Disease. 7th ed. McGraw-Hill Companies. New York, NY; 1995:1451-60.

JOURNAL ARTICLES

Peters V, Morath M, Mack M, et al. Formation of 3-hydroxyglutaric acid in glutaric aciduria type I: in vitro participation of medium chain acyl-CoA dehydrogenase. JIMD Rep. 2019;47(1):30-34. Published 2019 Mar 26. doi:10.1002/jmd2.12026

Boy N, Mengler K, Thimm E, et al Newborn screening: A disease-changing intervention for glutaric aciduria type 1. Ann Neurol. 2018 May;83(5):970-979. doi: 10.1002/ana.25233. Epub 2018 Apr 30.

Boy N, Mühlhausen C, Maier EM, et al. Proposed recommendations for diagnosing and managing individuals with glutaric aciduria type I: second revision. J Inherit Metab Dis. 2017 Jan;40(1):75-101. doi: 10.1007/s10545-016-9999-9. Epub 2016 Nov 16. Review. PubMed PMID: 27853989.select

Mosaeilhy A, Mohamed MM, C GPD, et al. Genotype-phenotype correlation in 18 Egyptian patients with glutaric acidemia type I. Metab Brain Dis. 2017 Oct;32(5):1417-1426. doi: 10.1007/s11011-017-0006-4. Epub 2017 Apr 7. PubMed PMID: 28389991.

Hedlund GL, Longo N, Pasquali M. Glutaric acidemia type 1. Am J Med Genet C Semin Med Genet. 2006 May 15;142C(2):86-94. doi: 10.1002/ajmg.c.30088. Review. PubMed PMID: 16602100; PubMed Central PMCID: PMC2556991.

Bähr O, Mader I, Zschocke J, Dichgans J, Schulz JB. Adult onset glutaric aciduria type I presenting with a leukoencephalopathy. Neurology. 2002 Dec 10;59(11):1802-4. doi: 10.1212/01.wnl.0000036616.11962.3c. PubMed PMID: 12473778.

Kölker S, Ramaekers VT, Zschocke J, Hoffmann GF. Acute encephalopathy despite early therapy in a patient with homozygosity for E365K in the glutaryl-coenzyme A dehydrogenase gene. J Pediatr. 2001 Feb;138(2):277-9. doi: 10.1067/mpd.2001.110303. PubMed PMID: 11174631.select

Busquets C, Coll MJ, Merinero B, et al. Prenatal molecular diagnosis of glutaric aciduria type I by direct mutation analysis. Prenat Diagn. 2000 Sep;20(9):761-4. PubMed PMID: 11015709.

Kafil-Hussain NA, Monavari A, Bowell R, Thornton P, Naughten E, O'Keefe M. Ocular findings in glutaric aciduria type 1. J Pediatr Ophthalmol Strabismus. 2000 Sep-Oct;37(5):289-93. PubMed PMID: 11020111.

Zafeiriou DI, Zschocke J, Augoustidou-Savvopoulou P, et al. Atypical and variable clinical presentation of glutaric aciduria type I. Neuropediatrics. 2000 Dec;31(6):303-6. doi: 10.1055/s-2000-12943. PubMed PMID: 11508549.

Baric I, Wagner L, Feyh P, Liesert M, Buckel W, Hoffmann GF. Sensitivity and specificity of free and total glutaric acid and 3-hydroxyglutaric acid measurements by stable-isotope dilution assays for the diagnosis of glutaric aciduria type I. J Inherit Metab Dis.1999 Dec;22(8):867-81. PubMed PMID: 10604139.

Hoffmann GF, Zschocke J. Glutaric aciduria type I: from clinical, biochemical and molecular diversity to successful therapy. J Inherit Metab Dis. 1999 Jun;22(4):381-91. Review. PubMed PMID: 10407775

Naylor EW, Chace DH. Automated tandem mass spectrometry for mass newborn screening for disorders in fatty acid, organic acid, and amino acid metabolism. J Child Neurol. 1999 Nov;14 Suppl 1:S4-8. doi: 10.1177/0883073899014001021. PubMed PMID: 10593560.

INTERNET

Genetic Home Reference. Glutaric Acidemia Type I. U.S. The National Library of Medicine. Last updated March 2007. https://ghr.nlm.nih.gov/condition/glutaric-acidemia-type-i. Accessed April 12, 2019.

McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). The Johns Hopkins University. Glutaric Acidemia I. Entry Number; 231670. 08/09/2017. https://www.omim.org/entry/231670 Accessed April 12, 2019.

Save Babies Through Screening. Glutaric acidemia Type I (GA-I). Last updated March 02, 2016. https://www.newbornscreening.info/Parents/organicaciddisorders/GA1.html Accessed April 12, 2019.

 

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Glutaric Acid-Mediated Apoptosis in Primary Striatal Neurons
Fengyan Tian,1,2 Xi Fu,1 Jinzhi Gao,1 Yanqin Ying,1 Ling Hou,1 Yan Liang,1 Qin Ning,3 and Xiaoping Luo1
1Department of Pediatrics, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, China
2Department of Pediatrics, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, China
3Laboratory of Infectious Immunology, Department of Infectious Disease, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, China

 

Received 12 February 2014; Revised 20 April 2014; Accepted 21 April 2014; Published 12 May 2014

Academic Editor: Janet K. Kern

Copyright © 2014 Fengyan Tian et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

 

Abstract
Glutaric acid (GA) has been implicated in the mechanism of neurodegeneration in glutaric aciduria type I. In the present study, the potential cytotoxic effects of GA (0.1~50 mM for 24~96 h) were examined in cultured primary rat striatal neurons. Results showed increase in the number of cells labeled by annexin-V or with apoptotic features shown by Hoechst/PI staining and transmission electron microscopy (TEM) and upregulation of the expression of mRNA as well as the active protein fragments caspase 3, suggesting involvement of the caspase 3-dependent apoptotic pathway in GA-induced striatal neuronal death. This effect was in part suppressed by the N-methyl-D-aspartate (NMDA) receptor antagonist MK-801 but not the α-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) antagonist 6-cyano-7-nitroquinoxalone-2,3-dione (CNQX). Thus, GA may trigger neuronal damage partially through apoptotic pathway and via activation of NMDA receptors in cultured primary striatal neurons.

 

 

1. Introduction
Glutaric aciduria type I (GA I) is an autosomal recessive organic aciduria caused by mutations in the gene encoding glutaryl-CoA dehydrogenase (GCDH). Patients are at risk of developing an irreversible dystonia and/or dyskinesia movement disorders as a sequence of selective destruction of striatal neurons following acute encephalopathic crises triggered by intercurrent catabolic situations such as infectious diseases, fever, vomiting, diarrhea, or immunization. The acute episodes of encephalopathy usually occurred within the first 3 years of life [1]. The main pathomorphological findings are frontotemporal atrophy, basal ganglia lesions, white matter disease, and postsynaptic vacuolization of neurons [2]. However, the clinical phenotype has no relationship to the biochemical phenotype or the genotype [1, 3].

 

 

Figure 8: Apoptosis examined by TEM. (a) Striatal neuron with normal ultrastructure. (b) Typical apoptotic cell (intermediate-late stage) with shrunken nucleus and condensed chromatin.
3.6. Effect of Glutaric Acid on Caspase 3 Transcript and Protein Levels
Quantitative RT-PCR was performed to monitor mRNA expression of the apoptotic executioner caspase 3 (Figure 9(a)). The comparative method was used to analyse relative expression levels. Caspase 3 mRNA expression at 6 hours after treatment with 1, 10, 25, and 50 mM GA was upregulated about 1.40-fold, 1.67-fold, and 1.95-fold, respectively, compared to control. Thus GA might induce apoptosis via caspase 3 activation.

 

 

fig9
Figure 9: Changes in the expression of caspase 3. (a) Kinetics of caspase 3 mRNA expression. Note: cells treated with 1, 10, 25, and 50 mM GA were compared with normal control cells incubated for the same treatment periods, respectively. and , compared to normal control cells incubated 1 h; and , compared to normal control cells incubated 6 h; and , compared to normal control cells incubated 12 h. (b) Changes in the expression of caspase 3 proteins in striatal neurons treated with 30 mM GA. Primary cultures were exposed to 30 mM GA with or without antagonist preincubation for 24 h. Caspase 3 (precursor and active fragment) levels increased after GA incubation. The increase in active fragment of caspase 3 was partly reduced by addition of MK-801 but not CNQX.
Western blot analysis of caspase 3 (Figure 9(b)) indicated that 30 mM GA increased levels of the precursor protein and active fragments of caspase 3 (Mr 35 and 17 kDa protein band) relative to the normal control. MK-801 but not CNQX lowered the GA-induced active caspase 3 changes in protein level, which suggested that MK-801 at least partly protected the striatal neuronal cells against GA-induced apoptosis.

 

 

4. Discussion
Glutaric aciduria type I is an autosomal recessive disorder characterized by high levels of GA, 3-hydroxyglutaric acid (3-OHGA), glutaconic acid, and glutaryl-CoA in body fluids as well as degenerative changes in the striatal and frontotemporal cortical neurons. A deficiency of cerebral GCDH activity is attributed to the development of neurological damage in GA I patients. However, the comprehension of the degeneration mechanism in the basal ganglia still remains partial.

 

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