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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-23 05:44:21 UTC
HMDB IDHMDB0000661
Secondary Accession Numbers
  • HMDB00661
Metabolite Identification
Common NameGlutaric acid
DescriptionGlutaric 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 ).
Structure
Data?1563860661
Synonyms
ValueSource
1,3-Propanedicarboxylic acidChEBI
1,5-Pentanedioic acidChEBI
GlutarsaeureChEBI
Pentanedioic acidChEBI
1,3-PropanedicarboxylateGenerator
1,5-PentanedioateGenerator
PentanedioateGenerator
GlutarateGenerator
PentandioateHMDB
Pentandioic acidHMDB
Chemical FormulaC5H8O4
Average Molecular Weight132.1146
Monoisotopic Molecular Weight132.042258744
IUPAC Namepentanedioic acid
Traditional Nameglutaric acid
CAS Registry Number110-94-1
SMILES
OC(=O)CCCC(O)=O
InChI Identifier
InChI=1S/C5H8O4/c6-4(7)2-1-3-5(8)9/h1-3H2,(H,6,7)(H,8,9)
InChI KeyJFCQEDHGNNZCLN-UHFFFAOYSA-N
Chemical Taxonomy
Description belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassDicarboxylic acids and derivatives
Direct ParentDicarboxylic acids and derivatives
Alternative Parents
Substituents
  • Fatty acid
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Role

Indirect biological role:

Industrial application:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point95.8 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility1600 mg/mLNot Available
LogP-0.29HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility56 g/LALOGPS
logP-0.25ALOGPS
logP0.046ChemAxon
logS-0.37ALOGPS
pKa (Strongest Acidic)3.76ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity28.14 m³·mol⁻¹ChemAxon
Polarizability12.17 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0f6t-2940000000-9f099473c4a6eb94d4beJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0002-1920000000-4ade63738a1c00460a63JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0002-1910000000-743df9571fee7baa3417JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-00fs-9710000000-ba958e52b1424e5d4840JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0cgj-3930000000-298fe512c6b58220ee33JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-052o-9000000000-663deab1ce6413c4859dJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-0920000000-56bce366cac24cd6773fJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0f6t-2940000000-9f099473c4a6eb94d4beJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-1920000000-4ade63738a1c00460a63JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-1910000000-743df9571fee7baa3417JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00fs-9710000000-ba958e52b1424e5d4840JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0cgj-3930000000-298fe512c6b58220ee33JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-86fbcf8f1c72fbb2ffd0JSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-01q3-9200000000-fcbc36fd55a7945d3f25JSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-05g3-9610000000-07394eb05b518a4e3bf4JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, N/A (Annotated)splash10-0019-9500000000-9db56ca3a541f0ce07d1JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, N/A (Annotated)splash10-052r-9100000000-991f69d0892976558f85JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, N/A (Annotated)splash10-0536-9300000000-20fb2c8dd136e4924acaJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - EI-B (Unknown) , Positivesplash10-052o-9000000000-663deab1ce6413c4859dJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-001i-0900000000-8eb90c15915f014713acJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-000i-9200000000-2ffdc08c49952e135a86JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-000i-9000000000-8884b857c9582288f35aJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-052f-9000000000-7d2c0333bdce4dbabbc6JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0006-9000000000-9ab2a948083302641628JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-000i-9400000000-c5ec3115706f6041b868JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-001i-0900000000-8eb90c15915f014713acJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-000i-9200000000-2ffdc08c49952e135a86JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-000i-9000000000-8884b857c9582288f35aJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-052f-9000000000-7d2c0333bdce4dbabbc6JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9000000000-9ab2a948083302641628JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , negativesplash10-000i-9400000000-c5ec3115706f6041b868JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00lr-3900000000-e068e1a5fced6447f9efJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-01c9-9400000000-ed0953fcaa050d721ed0JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-002f-9000000000-9a285d856528d3c30034JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-001i-1900000000-2ccd8e2a515cf328e787JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-001i-6900000000-0ea4829580fe919d05a0JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9000000000-f1b2458f574a96cbcb83JSpectraViewer | MoNA
MSMass Spectrum (Electron Ionization)splash10-052o-9000000000-f308daa4e82c16019e0dJSpectraViewer | MoNA
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR13C NMR SpectrumNot AvailableJSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableJSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.8 (0.0-1.8) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0 - 1.8 uMNewborn (0-30 days old)MaleNormal details
BloodDetected and Quantified0 uMChildren (1 - 13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0 - 1 uMNewborn (0-30 days old)MaleNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.28 ( 0.18-0.63) uMNot SpecifiedNot SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1 uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.93 +/- 0.38 uMNot SpecifiedNot SpecifiedNormal details
FecesDetected but not Quantified Infant (0-1 year old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Not Specified
Normal
details
FecesDetected and Quantified189.154 +/- 125.421 nmol/g wet fecesNot SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
SalivaDetected and Quantified0.0770 +/- 0.0431 uMAdult (>18 years old)Both
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected and Quantified0.501 +/- 1.36 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.546 +/- 0.712 uMAdult (>18 years old)Both
Normal
    • Sugimoto et al. (...
details
UrineDetected and Quantified<3.392 umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified<101.754 umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified<10 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified3.0 +/- 1.2 umol/mmol creatinineAdolescent (13-18 years old)FemaleNormal details
UrineDetected and Quantified<13 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified0.96-1.8 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified5.5 (0.0-11.0) umol/mmol creatinineInfant (0-1 year old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified3.58 +/- 0.87 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified<5 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified1.3 (0.6-2.6) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<9.33 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified<3.392 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified<51.346 umol/mmol creatinineInfant (0-1 year old)MaleNormal details
UrineDetected and Quantified0.100-14 umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
UrineDetected and Quantified5.800 +/- 3.800 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified0.87-1.4 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified2.1 (0.7-3.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified11.906 +/- 7.265 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified1.08 +/- 1.24 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
UrineDetected and Quantified9.4 (2.9 - 19.1) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified12.5 (0.1 - 14.7) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified1.4 (0.5-2.3) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2.4 (0.1 - 4.8) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified2.5 (0.7 - 5.2) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified4.599 +/- 5.212 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified12 uMNewborn (0-30 days old)MaleGlutaric aciduria I details
Cerebrospinal Fluid (CSF)Detected and Quantified39.74 uMNot AvailableNot Specified
Glutaryl-CoA dehydrogenase deficiency (GDHD)
details
Cerebrospinal Fluid (CSF)Detected and Quantified43 uMNewborn (0-30 days old)MaleGlutaric aciduria I details
FecesDetected but not Quantified Adult (>18 years old)Both
Irritable bowel syndrome
details
FecesDetected but not Quantified Adult (>18 years old)Both
Ulcerative colitis
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Cryptosporidium infection
details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified3.392-44.0934 umol/mmol creatinineNewborn (0-30 days old)FemaleShort Chain Acyl-Coa Dehydrogenase Deficiency details
UrineDetected and Quantified4.522-9.0448 umol/mmol creatinineNewborn (0-30 days old)FemaleShort Chain Acyl-Coa Dehydrogenase Deficiency details
UrineDetected and Quantified290-1460 umol/mmol creatinineChildren (1-13 years old)BothGlutaric aciduria type III details
UrineDetected and Quantified557 umol/mmol creatinineInfant (0-1 year old)Male3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency details
UrineDetected and Quantified5.46 +/- 2.12 umol/mmol creatinineChildren (1-13 years old)BothMalnutrition (type kwashiorkor and marasmus) details
UrineDetected and Quantified1274.9 umol/mmol creatinineNewborn (0-30 days old)Male3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
UrineDetected and Quantified3.4 +/- 1.9 umol/mmol creatinineAdolescent (13-18 years old)FemaleAnorexia nervosa details
UrineDetected and Quantified5.135 umol/mmol creatinineInfant (0-1 year old)MaleFumarase deficiency details
UrineDetected and Quantified40 umol/mmol creatinineInfant (0-1 year old)MaleMalonyl-Coa decarboxylase deficiency details
UrineDetected and Quantified96.81 umol/mmol creatinineAdolescent (13-18 years old)FemaleGlutaric aciduria I details
UrineDetected and Quantified1274.9 umol/mmol creatinineNewborn (0-30 days old)Male3-hydroxy-3-methylglutaric- coenzyme A lyase deficiency details
UrineDetected and Quantified7870 umol/mmol creatinineNewborn (0-30 days old)MaleGlutaric aciduria I details
UrineDetected and Quantified0-18.200 umol/mmol creatinineInfant (0-1 year old)Both
Short chain acyl-CoA Dehydrogenase deficiency (SCAD)
details
UrineDetected and Quantified0-9.0448 umol/mmol creatinineInfant (0-1 year old)Female
Short chain acyl-CoA dehydrogenase deficiency (SCAD)
details
UrineDetected but not Quantified Adult (>18 years old)BothBladder cancer details
UrineDetected and Quantified15.232 +/- 15.988 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified4.375 +/- 2.82 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified4.162 +/- 2.373 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
Glutaric aciduria I
  1. 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 ]
  2. 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 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Glutaryl-CoA dehydrogenase deficiency (GDHD)
  1. 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
  1. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
Ulcerative colitis
  1. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  3. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Anorexia nervosa
  1. Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed:10197568 ]
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
Fumarase deficiency
  1. Bastug O, Kardas F, Ozturk MA, Halis H, Memur S, Korkmaz L, Tag Z, Gunes T: A rare cause of opistotonus; fumaric aciduria: The first case presentation in Turkey. Turk Pediatri Ars. 2014 Mar 1;49(1):74-6. doi: 10.5152/tpa.2014.442. eCollection 2014 Mar. [PubMed:26078636 ]
Malonyl-Coa decarboxylase deficiency
  1. Haan EA, Scholem RD, Croll HB, Brown GK: Malonyl coenzyme A decarboxylase deficiency. Clinical and biochemical findings in a second child with a more severe enzyme defect. Eur J Pediatr. 1986 Apr;144(6):567-70. [PubMed:3709568 ]
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
  1. Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ]
Short Chain Acyl-Coa Dehydrogenase Deficiency
  1. Bhala A, Willi SM, Rinaldo P, Bennett MJ, Schmidt-Sommerfeld E, Hale DE: Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1995 Jun;126(6):910-5. [PubMed:7776094 ]
  2. Amendt BA, Greene C, Sweetman L, Cloherty J, Shih V, Moon A, Teel L, Rhead WJ: Short-chain acyl-coenzyme A dehydrogenase deficiency. Clinical and biochemical studies in two patients. J Clin Invest. 1987 May;79(5):1303-9. [PubMed:3571488 ]
3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency
  1. Conboy E, Vairo F, Schultz M, Agre K, Ridsdale R, Deyle D, Oglesbee D, Gavrilov D, Klee EW, Lanpher B: Mitochondrial 3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency: Unique Presenting Laboratory Values and a Review of Biochemical and Clinical Features. JIMD Rep. 2017 Oct 14. doi: 10.1007/8904_2017_59. [PubMed:29030856 ]
Glutaric aciduria type III
  1. 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 ]
Associated OMIM IDs
  • 114500 (Colorectal cancer)
  • 606788 (Anorexia nervosa)
  • 610247 (Eosinophilic esophagitis)
  • 606812 (Fumarase deficiency)
  • 248360 (Malonyl-Coa decarboxylase deficiency)
  • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 605911 (3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency)
  • 231690 (Glutaric aciduria type III)
DrugBank IDDB03553
Phenol Explorer Compound IDNot Available
FoodDB IDFDB001477
KNApSAcK IDC00001184
Chemspider ID723
KEGG Compound IDC00489
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkGlutaric_acid
METLIN ID3254
PubChem Compound743
PDB IDNot Available
ChEBI ID17859
References
Synthesis ReferencePetrova, 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)
General References
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. 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 ]
  9. 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 ]
  10. Singh I: Biochemistry of peroxisomes in health and disease. Mol Cell Biochem. 1997 Feb;167(1-2):1-29. [PubMed:9059978 ]
  11. 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 ]
  12. 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 ]
  13. 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 ]
  14. 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. Epub 2018 Jul 10. [PubMed:30143200 ]

Transporters

General function:
Involved in transporter activity
Specific function:
Mediates saturable uptake of estrone sulfate, dehydroepiandrosterone sulfate and related compounds
Gene Name:
SLC22A11
Uniprot ID:
Q9NSA0
Molecular weight:
59970.9
References
  1. Cha SH, Sekine T, Kusuhara H, Yu E, Kim JY, Kim DK, Sugiyama Y, Kanai Y, Endou H: Molecular cloning and characterization of multispecific organic anion transporter 4 expressed in the placenta. J Biol Chem. 2000 Feb 11;275(6):4507-12. [PubMed:10660625 ]
General function:
Involved in ion transmembrane transporter activity
Specific function:
Involved in the renal elimination of endogenous and exogenous organic anions. Functions as organic anion exchanger when the uptake of one molecule of organic anion is coupled with an efflux of one molecule of endogenous dicarboxylic acid (glutarate, ketoglutarate, etc). Mediates the sodium-independent uptake of 2,3-dimercapto-1-propanesulfonic acid (DMPS). Mediates the sodium-independent uptake of p- aminohippurate (PAH), ochratoxin (OTA), acyclovir (ACV), 3'-azido- 3-'deoxythymidine (AZT), cimetidine (CMD), 2,4-dichloro- phenoxyacetate (2,4-D), hippurate (HA), indoleacetate (IA), indoxyl sulfate (IS) and 3-carboxy-4-methyl-5-propyl-2- furanpropionate (CMPF), cidofovir, adefovir, 9-(2- phosphonylmethoxyethyl) guanine (PMEG), 9-(2- phosphonylmethoxyethyl) diaminopurine (PMEDAP) and edaravone sulfate. PAH uptake is inhibited by p- chloromercuribenzenesulphonate (PCMBS), diethyl pyrocarbonate (DEPC), sulindac, diclofenac, carprofen, glutarate and okadaic acid. PAH uptake is inhibited by benzothiazolylcysteine (BTC), S-chlorotrifluoroethylcysteine (CTFC), cysteine S-conjugates S-dichlorovinylcysteine (DCVC), furosemide, steviol, phorbol 12-myristate 13-acetate (PMA), calcium ionophore A23187, benzylpenicillin, furosemide, indomethacin, bumetamide, losartan, probenecid, phenol red, urate, and alpha-ketoglutarate
Gene Name:
SLC22A6
Uniprot ID:
Q4U2R8
Molecular weight:
61815.8
References
  1. Cihlar T, Ho ES: Fluorescence-based assay for the interaction of small molecules with the human renal organic anion transporter 1. Anal Biochem. 2000 Jul 15;283(1):49-55. [PubMed:10929807 ]
  2. Lu R, Chan BS, Schuster VL: Cloning of the human kidney PAH transporter: narrow substrate specificity and regulation by protein kinase C. Am J Physiol. 1999 Feb;276(2 Pt 2):F295-303. [PubMed:9950961 ]
  3. Uwai Y, Okuda M, Takami K, Hashimoto Y, Inui K: Functional characterization of the rat multispecific organic anion transporter OAT1 mediating basolateral uptake of anionic drugs in the kidney. FEBS Lett. 1998 Nov 6;438(3):321-4. [PubMed:9827570 ]
  4. Aslamkhan A, Han YH, Walden R, Sweet DH, Pritchard JB: Stoichiometry of organic anion/dicarboxylate exchange in membrane vesicles from rat renal cortex and hOAT1-expressing cells. Am J Physiol Renal Physiol. 2003 Oct;285(4):F775-83. Epub 2003 Jul 1. [PubMed:12837685 ]
General function:
Involved in transmembrane transport
Specific function:
Mediates sodium-independent multispecific organic anion transport. Transport of prostaglandin E2, prostaglandin F2, tetracycline, bumetanide, estrone sulfate, glutarate, dehydroepiandrosterone sulfate, allopurinol, 5-fluorouracil, paclitaxel, L-ascorbic acid, salicylate, ethotrexate, and alpha- ketoglutarate
Gene Name:
SLC22A7
Uniprot ID:
Q9Y694
Molecular weight:
60025.0
References
  1. 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 ]