Hmdb loader
Survey
You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:15:08 UTC
HMDB IDHMDB0000752
Secondary Accession Numbers
  • HMDB00752
Metabolite Identification
Common NameMethylglutaric acid
DescriptionMethylglutaric acid, also known as b-methylglutarate or 3-methylpentanedioate, belongs to the class of organic compounds known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present. Based on a literature review a significant number of articles have been published on Methylglutaric acid.
Structure
Data?1676999708
Synonyms
ValueSource
beta-Methylglutaric acidChEBI
b-MethylglutarateGenerator
b-Methylglutaric acidGenerator
beta-MethylglutarateGenerator
Β-methylglutarateGenerator
Β-methylglutaric acidGenerator
MethylglutarateGenerator
3-Methyl-glutarateHMDB
3-Methyl-glutaric acidHMDB
3-MethylglutarateHMDB
3-Methylglutaric acidHMDB
3-MethylpentanedioateHMDB
3-Methylpentanedioic acidHMDB
b-Methyl-glutaric acidHMDB
beta-Methyl-glutaric acidHMDB
Chemical FormulaC6H10O4
Average Molecular Weight146.1412
Monoisotopic Molecular Weight146.057908808
IUPAC Name3-methylpentanedioic acid
Traditional Namemethylglutaric acid
CAS Registry Number626-51-7
SMILES
CC(CC(O)=O)CC(O)=O
InChI Identifier
InChI=1S/C6H10O4/c1-4(2-5(7)8)3-6(9)10/h4H,2-3H2,1H3,(H,7,8)(H,9,10)
InChI KeyXJMMNTGIMDZPMU-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentMethyl-branched fatty acids
Alternative Parents
Substituents
  • Methyl-branched fatty acid
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point80 - 82 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Not Available122.881http://allccs.zhulab.cn/database/detail?ID=AllCCS00000211
Predicted Molecular Properties
PropertyValueSource
Water Solubility36.9 g/LALOGPS
logP0.33ALOGPS
logP0.33ChemAxon
logS-0.6ALOGPS
pKa (Strongest Acidic)3.91ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity32.69 m³·mol⁻¹ChemAxon
Polarizability13.88 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+132.68231661259
DarkChem[M-H]-129.51431661259
AllCCS[M+H]+133.56632859911
AllCCS[M-H]-129.96432859911
DeepCCS[M+H]+124.94530932474
DeepCCS[M-H]-121.46130932474
DeepCCS[M-2H]-158.28530932474
DeepCCS[M+Na]+133.50530932474
AllCCS[M+H]+133.632859911
AllCCS[M+H-H2O]+129.532859911
AllCCS[M+NH4]+137.332859911
AllCCS[M+Na]+138.432859911
AllCCS[M-H]-130.032859911
AllCCS[M+Na-2H]-132.232859911
AllCCS[M+HCOO]-134.832859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
Methylglutaric acidCC(CC(O)=O)CC(O)=O2153.1Standard polar33892256
Methylglutaric acidCC(CC(O)=O)CC(O)=O1068.5Standard non polar33892256
Methylglutaric acidCC(CC(O)=O)CC(O)=O1317.0Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Methylglutaric acid,1TMS,isomer #1CC(CC(=O)O)CC(=O)O[Si](C)(C)C1357.0Semi standard non polar33892256
Methylglutaric acid,2TMS,isomer #1CC(CC(=O)O[Si](C)(C)C)CC(=O)O[Si](C)(C)C1425.0Semi standard non polar33892256
Methylglutaric acid,1TBDMS,isomer #1CC(CC(=O)O)CC(=O)O[Si](C)(C)C(C)(C)C1599.1Semi standard non polar33892256
Methylglutaric acid,2TBDMS,isomer #1CC(CC(=O)O[Si](C)(C)C(C)(C)C)CC(=O)O[Si](C)(C)C(C)(C)C1865.5Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - Methylglutaric acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0002-0910000000-81203203246ac374e1aa2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Methylglutaric acid GC-MS (2 TMS)splash10-00xr-2930000000-8a7588ab0eaf67584f922014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Methylglutaric acid EI-B (Non-derivatized)splash10-0002-0930000000-5140c0f39766a00d84822017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Methylglutaric acid EI-B (Non-derivatized)splash10-0002-0930000000-fbddcb97da0458138e6b2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Methylglutaric acid GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-81203203246ac374e1aa2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Methylglutaric acid GC-MS (Non-derivatized)splash10-00xr-2930000000-8a7588ab0eaf67584f922017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Methylglutaric acid GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-1a70e49844a70861269a2017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Methylglutaric acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-000i-9400000000-4f63fb4e9602cbd175642017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Methylglutaric acid GC-MS (2 TMS) - 70eV, Positivesplash10-00dr-9320000000-32ac86c797d0bc1d6ae42017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Methylglutaric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Methylglutaric acid GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Methylglutaric acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Methylglutaric acid GC-MS (TBDMS_2_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
MSMass Spectrum (Electron Ionization)splash10-0frf-9100000000-a7e54dd57900b7dde6012014-09-20Not AvailableView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-0udi-0900000000-eb243e6803451ac1e4842012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-0a4i-9000000000-f5d1e90d94d8c01d64132012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-0aor-9200000000-5a9c1dfa7ad59a2bb3092012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid , negative-QTOFsplash10-0udi-0900000000-1b870c5514c1893d28a02017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid , positive-QTOFsplash10-000i-6900000000-a6e3d73c4975ca021d592017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid 35V, Negative-QTOFsplash10-0udi-0900000000-a8aaab100afd68e0ee182021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid 35V, Negative-QTOFsplash10-0udi-0900000000-77ddf18d229a8d1ff0f82021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid 20V, Negative-QTOFsplash10-0udi-3900000000-8ef7eec833de94a948562021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid 10V, Negative-QTOFsplash10-0udi-0900000000-a18325730b3c402daa892021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid 40V, Negative-QTOFsplash10-0006-9000000000-30a27debcc4654b89e5f2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid 20V, Negative-QTOFsplash10-0udi-5900000000-d1e2a84b0595aba908662021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid 10V, Negative-QTOFsplash10-0udi-0900000000-be33f7d5039cfbdd408b2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Methylglutaric acid 40V, Negative-QTOFsplash10-0006-9000000000-ad1c751d3305adf1a42e2021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 10V, Positive-QTOFsplash10-004j-1900000000-0d7094ff6083f2cbfb112017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 20V, Positive-QTOFsplash10-0ftu-9400000000-3e6419aef7a791fa14442017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 40V, Positive-QTOFsplash10-0006-9000000000-a7c4a49f42f193d900b02017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 10V, Negative-QTOFsplash10-0002-1900000000-1eb2402c520f7d0097432017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 20V, Negative-QTOFsplash10-0f92-2900000000-32ccc4d90c40abb613442017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 40V, Negative-QTOFsplash10-0a4i-9200000000-5b5c0b72c3ec914ca4e72017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 10V, Negative-QTOFsplash10-0udi-1900000000-313ba34cea08a11aed202021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 20V, Negative-QTOFsplash10-0ue9-9700000000-8fc6c8a4483eb77abb352021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 40V, Negative-QTOFsplash10-0006-9000000000-6af719f8e3b08b963f742021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 10V, Positive-QTOFsplash10-0ugi-4900000000-64cf0487d11a501c31e42021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 20V, Positive-QTOFsplash10-0lzc-9300000000-a18f183c17700eee0d0e2021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Methylglutaric acid 40V, Positive-QTOFsplash10-0006-9000000000-652731b0711cef7d7b412021-09-24Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)2023-02-03FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue Locations
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot QuantifiedNot AvailableNot Available
Normal
      Not Available
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified<5 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified0.5 +/- 0.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified1.0 - 6.5 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified0.75-1.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified<500 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified<7 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified0-1 umol/mmol creatinineInfant (0-1 year old)Male
Normal
details
UrineDetected and Quantified0.54-0.80 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0-1.3 umol/mmol creatinineInfant (0-1 year old)Female
Normal
details
UrineDetected and Quantified0-1.5 umol/mmol creatinineInfant (0-1 year old)Male
Normal
details
UrineDetected and Quantified<3.9 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.6 (1.3-2.0) umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified0.0-1.1 umol/mmol creatinineInfant (0-1 year old)Female
Normal
details
UrineDetected and Quantified<5 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified0.1 (0.1-9.7) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified0.1 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified1.0 (0.1-2.8) umol/mmol creatinineChildren (1-13 years old)Both
Normal
details
UrineDetected and Quantified1.1 (0.1-2.0) umol/mmol creatinineChildren (1-13 years old)Both
Normal
details
UrineDetected and Quantified0.6 (0.1-2.1) umol/mmol creatinineAdolescent (13-18 years old)Both
Normal
details
UrineDetected and Quantified3.1 (2.6-3.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified348 umol/mmol creatinineNewborn (0-30 days old)Male3-hydroxy-3-methylglutaric- coenzyme A lyase deficiency details
UrineDetected and Quantified622 umol/mmol creatinineAdult (>18 years old)Female3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
UrineDetected and Quantified12-15 umol/mmol creatinineAdult (>18 years old)Female3-methylglutaconic aciduria type I details
UrineDetected and Quantified1400 umol/mmol creatinineChildren (1-13 years old)Male3-Methylglutaconic Aciduria (Type V) details
UrineDetected and Quantified348 umol/mmol creatinineNewborn (0-30 days old)Male3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
UrineDetected and Quantified622 umol/mmol creatinineAdult (>18 years old)Female3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
UrineDetected and Quantified3.5 (0.0-7.0) umol/mmol creatinineAdult (>18 years old)Both3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
    • MetaGene: Metabol...
details
UrineDetected and Quantified507.0 (14.0-1000.0) umol/mmol creatinineChildren (1-13 years old)Both3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
    • MetaGene: Metabol...
details
Associated Disorders and Diseases
Disease References
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. 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 ]
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 ]
  2. Bischof F, Nagele T, Wanders RJ, Trefz FK, Melms A: 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in an adult with leukoencephalopathy. Ann Neurol. 2004 Nov;56(5):727-30. [PubMed:15505778 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
3-Methylglutaconic aciduria type I
  1. Engelke UF, Kremer B, Kluijtmans LA, van der Graaf M, Morava E, Loupatty FJ, Wanders RJ, Moskau D, Loss S, van den Bergh E, Wevers RA: NMR spectroscopic studies on the late onset form of 3-methylglutaconic aciduria type I and other defects in leucine metabolism. NMR Biomed. 2006 Apr;19(2):271-8. [PubMed:16541463 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
3-Methylglutaconic Aciduria type V
  1. Ojala T, Polinati P, Manninen T, Hiippala A, Rajantie J, Karikoski R, Suomalainen A, Tyni T: New mutation of mitochondrial DNAJC19 causing dilated and noncompaction cardiomyopathy, anemia, ataxia, and male genital anomalies. Pediatr Res. 2012 Oct;72(4):432-7. doi: 10.1038/pr.2012.92. Epub 2012 Jul 13. [PubMed:22797137 ]
Associated OMIM IDs
  • 114500 (Colorectal cancer)
  • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 250950 (3-Methylglutaconic aciduria type I)
  • 610198 (3-Methylglutaconic Aciduria type V)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022224
KNApSAcK IDC00001187
Chemspider ID11781
KEGG Compound IDNot Available
BioCyc IDCPD-516
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID5720
PubChem Compound12284
PDB IDNot Available
ChEBI ID68566
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00013434
Good Scents IDNot Available
References
Synthesis ReferenceKomppa, Gust. b-Methylglutaric acid. Ann. acad. sci. Fennicae (1930), 30(Ser. A;No. 9), 5 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. 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 ]
  2. 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 ]
  3. Truscott RJ, Halpern B, Wysocki SJ, Hahnel R, Wilcken B: Studies on a child suspected of having a dficiency in 3-hydroxy-3-methylglutaryl-Co A lyase. Clin Chim Acta. 1979 Jul 2;95(1):11-16. [PubMed:509721 ]
  4. Sheffer RN, Zlotogora J, Elpeleg ON, Raz J, Ben-Ezra D: Behr's syndrome and 3-methylglutaconic aciduria. Am J Ophthalmol. 1992 Oct 15;114(4):494-7. [PubMed:1384336 ]
  5. Duran M, Beemer FA, Tibosch AS, Bruinvis L, Ketting D, Wadman SK: Inherited 3-methylglutaconic aciduria in two brothers--another defect of leucine metabolism. J Pediatr. 1982 Oct;101(4):551-4. [PubMed:6181239 ]