Hmdb loader
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:15:07 UTC
HMDB IDHMDB0000742
Secondary Accession Numbers
  • HMDB0000165
  • HMDB00165
  • HMDB00742
Metabolite Identification
Common NameHomocysteine
DescriptionHomocysteine (CAS: 454-29-5) is a sulfur-containing amino acid that arises during methionine metabolism. Although its concentration in plasma is only about 10 micromolar (uM), even moderate hyperhomocysteinemia is associated with an increased incidence of cardiovascular disease and Alzheimer's disease. Elevations in plasma homocysteine are commonly found as a result of vitamin deficiencies, polymorphisms of enzymes of methionine metabolism, and renal disease. It has been identified as a uremic toxin according to the European Uremic Toxin Working Group (PMID: 22626821 ). Pyridoxal, folic acid, riboflavin, and vitamin B(12) are all required for methionine metabolism, and deficiency of each of these vitamins result in elevated plasma homocysteine. A polymorphism of methylenetetrahydrofolate reductase (C677T), which is quite common in most populations with a homozygosity rate of 10-15 %, is associated with moderate hyperhomocysteinemia, especially in the context of marginal folate intake. Plasma homocysteine is inversely related to plasma creatinine in patients with renal disease. This is due to an impairment in homocysteine removal in renal disease. The role of these factors, and of modifiable lifestyle factors, in affecting methionine metabolism and in determining plasma homocysteine levels is discussed. Homocysteine is an independent cardiovascular disease (CVD) risk factor modifiable by nutrition and possibly exercise. Homocysteine was first identified as an important biological compound in 1932 and linked with human disease in 1962 when elevated urinary homocysteine levels were found in children with mental retardation. This condition, called homocystinuria, was later associated with premature occlusive CVD, even in children. These observations led to research investigating the relationship of elevated homocysteine levels and CVD in a wide variety of populations including middle age and elderly men and women with and without traditional risk factors for CVD (PMID: 17136938 , 15630149 ). Moreover, homocysteine is found to be associated with cystathionine beta-synthase deficiency, cystathioninuria, methylenetetrahydrofolate reductase deficiency, and sulfite oxidase deficiency, which are inborn errors of metabolism.
Structure
Data?1676999707
Synonyms
ValueSource
HcyChEBI
L-2-Amino-4-mercaptobutyric acidChEBI
L-2-Amino-4-mercaptobutyrateGenerator
2-Amino-4-mercaptobutyric acidHMDB
Homocysteine, L isomerHMDB
L-Isomer homocysteineHMDB
2 Amino 4 mercaptobutyric acidHMDB
Homocysteine, L-isomerHMDB
(S)-2-Amino-4-mercapto-butanoateHMDB
(S)-2-Amino-4-mercapto-butanoic acidHMDB
(S)-2-Amino-4-mercaptobutanoic acidHMDB
(S)-HomocysteineHMDB
2-Amino-4-mercapto-DL-butyrateHMDB
2-Amino-4-mercapto-DL-butyric acidHMDB
2-Amino-4-mercapto-L-butyric acidHMDB
2-Amino-4-mercapto-butanoateHMDB
2-Amino-4-mercapto-butanoic acidHMDB
2-Amino-4-mercapto-butyric acidHMDB
2-Amino-4-sulfanylbutanoateHMDB
2-Amino-4-sulfanylbutanoic acidHMDB
D,L-HomocysteineHMDB
DL-2-Amino-4-mercapto-butyric acidHMDB
DL-2-Amino-4-mercaptobutyric acidHMDB
DL-HomocysteineHMDB
Homo-cysHMDB
L-2-Amino-4-mercapto-butyric acidHMDB
L-HomocysteineHMDB
2-Amino-4-mercapto-butyrateHMDB
HomocysteineMeSH, KEGG
Chemical FormulaC4H9NO2S
Average Molecular Weight135.185
Monoisotopic Molecular Weight135.035399227
IUPAC Name(2S)-2-amino-4-sulfanylbutanoic acid
Traditional NameL-homocysteine
CAS Registry Number6027-13-0
SMILES
N[C@@H](CCS)C(O)=O
InChI Identifier
InChI=1S/C4H9NO2S/c5-3(1-2-8)4(6)7/h3,8H,1-2,5H2,(H,6,7)/t3-/m0/s1
InChI KeyFFFHZYDWPBMWHY-VKHMYHEASA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
Substituents
  • L-alpha-amino acid
  • Thia fatty acid
  • Fatty acid
  • Fatty acyl
  • Amino acid
  • Alkylthiol
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organopnictogen compound
  • Primary aliphatic amine
  • Organic oxygen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point232 - 233 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility148 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Astarita_neg119.330932474
[M-H]-Baker159.6230932474
[M+H]+Baker130.53130932474
Predicted Molecular Properties
PropertyValueSource
Water Solubility14.8 g/LALOGPS
logP-2.3ALOGPS
logP-2.6ChemAxon
logS-0.96ALOGPS
pKa (Strongest Acidic)2.46ChemAxon
pKa (Strongest Basic)9.41ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area63.32 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity32.94 m³·mol⁻¹ChemAxon
Polarizability13.54 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
DeepCCS[M+H]+127.16430932474
DeepCCS[M-H]-123.59830932474
DeepCCS[M-2H]-160.54630932474
DeepCCS[M+Na]+135.74830932474
AllCCS[M+H]+131.832859911
AllCCS[M+H-H2O]+127.732859911
AllCCS[M+NH4]+135.532859911
AllCCS[M+Na]+136.632859911
AllCCS[M-H]-130.232859911
AllCCS[M+Na-2H]-133.532859911
AllCCS[M+HCOO]-137.132859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
HomocysteineN[C@@H](CCS)C(O)=O2221.7Standard polar33892256
HomocysteineN[C@@H](CCS)C(O)=O1253.5Standard non polar33892256
HomocysteineN[C@@H](CCS)C(O)=O1627.8Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Homocysteine,1TMS,isomer #1C[Si](C)(C)OC(=O)[C@@H](N)CCS1357.5Semi standard non polar33892256
Homocysteine,1TMS,isomer #2C[Si](C)(C)SCC[C@H](N)C(=O)O1502.8Semi standard non polar33892256
Homocysteine,1TMS,isomer #3C[Si](C)(C)N[C@@H](CCS)C(=O)O1435.1Semi standard non polar33892256
Homocysteine,2TMS,isomer #1C[Si](C)(C)OC(=O)[C@@H](N)CCS[Si](C)(C)C1543.5Semi standard non polar33892256
Homocysteine,2TMS,isomer #1C[Si](C)(C)OC(=O)[C@@H](N)CCS[Si](C)(C)C1619.2Standard non polar33892256
Homocysteine,2TMS,isomer #1C[Si](C)(C)OC(=O)[C@@H](N)CCS[Si](C)(C)C2156.5Standard polar33892256
Homocysteine,2TMS,isomer #2C[Si](C)(C)N[C@@H](CCS)C(=O)O[Si](C)(C)C1479.9Semi standard non polar33892256
Homocysteine,2TMS,isomer #2C[Si](C)(C)N[C@@H](CCS)C(=O)O[Si](C)(C)C1490.3Standard non polar33892256
Homocysteine,2TMS,isomer #2C[Si](C)(C)N[C@@H](CCS)C(=O)O[Si](C)(C)C1729.0Standard polar33892256
Homocysteine,2TMS,isomer #3C[Si](C)(C)N[C@@H](CCS[Si](C)(C)C)C(=O)O1627.3Semi standard non polar33892256
Homocysteine,2TMS,isomer #3C[Si](C)(C)N[C@@H](CCS[Si](C)(C)C)C(=O)O1612.6Standard non polar33892256
Homocysteine,2TMS,isomer #3C[Si](C)(C)N[C@@H](CCS[Si](C)(C)C)C(=O)O2081.6Standard polar33892256
Homocysteine,2TMS,isomer #4C[Si](C)(C)N([C@@H](CCS)C(=O)O)[Si](C)(C)C1565.1Semi standard non polar33892256
Homocysteine,2TMS,isomer #4C[Si](C)(C)N([C@@H](CCS)C(=O)O)[Si](C)(C)C1499.3Standard non polar33892256
Homocysteine,2TMS,isomer #4C[Si](C)(C)N([C@@H](CCS)C(=O)O)[Si](C)(C)C1906.0Standard polar33892256
Homocysteine,3TMS,isomer #1C[Si](C)(C)N[C@@H](CCS[Si](C)(C)C)C(=O)O[Si](C)(C)C1651.6Semi standard non polar33892256
Homocysteine,3TMS,isomer #1C[Si](C)(C)N[C@@H](CCS[Si](C)(C)C)C(=O)O[Si](C)(C)C1676.4Standard non polar33892256
Homocysteine,3TMS,isomer #1C[Si](C)(C)N[C@@H](CCS[Si](C)(C)C)C(=O)O[Si](C)(C)C1755.7Standard polar33892256
Homocysteine,3TMS,isomer #2C[Si](C)(C)OC(=O)[C@H](CCS)N([Si](C)(C)C)[Si](C)(C)C1628.1Semi standard non polar33892256
Homocysteine,3TMS,isomer #2C[Si](C)(C)OC(=O)[C@H](CCS)N([Si](C)(C)C)[Si](C)(C)C1639.6Standard non polar33892256
Homocysteine,3TMS,isomer #2C[Si](C)(C)OC(=O)[C@H](CCS)N([Si](C)(C)C)[Si](C)(C)C1739.0Standard polar33892256
Homocysteine,3TMS,isomer #3C[Si](C)(C)SCC[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1790.4Semi standard non polar33892256
Homocysteine,3TMS,isomer #3C[Si](C)(C)SCC[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1750.6Standard non polar33892256
Homocysteine,3TMS,isomer #3C[Si](C)(C)SCC[C@@H](C(=O)O)N([Si](C)(C)C)[Si](C)(C)C1905.9Standard polar33892256
Homocysteine,4TMS,isomer #1C[Si](C)(C)OC(=O)[C@H](CCS[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1825.8Semi standard non polar33892256
Homocysteine,4TMS,isomer #1C[Si](C)(C)OC(=O)[C@H](CCS[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1805.5Standard non polar33892256
Homocysteine,4TMS,isomer #1C[Si](C)(C)OC(=O)[C@H](CCS[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1703.5Standard polar33892256
Homocysteine,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@@H](N)CCS1605.7Semi standard non polar33892256
Homocysteine,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)SCC[C@H](N)C(=O)O1738.9Semi standard non polar33892256
Homocysteine,1TBDMS,isomer #3CC(C)(C)[Si](C)(C)N[C@@H](CCS)C(=O)O1695.9Semi standard non polar33892256
Homocysteine,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@@H](N)CCS[Si](C)(C)C(C)(C)C2009.2Semi standard non polar33892256
Homocysteine,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@@H](N)CCS[Si](C)(C)C(C)(C)C2069.2Standard non polar33892256
Homocysteine,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@@H](N)CCS[Si](C)(C)C(C)(C)C2244.6Standard polar33892256
Homocysteine,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)N[C@@H](CCS)C(=O)O[Si](C)(C)C(C)(C)C1948.0Semi standard non polar33892256
Homocysteine,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)N[C@@H](CCS)C(=O)O[Si](C)(C)C(C)(C)C1925.4Standard non polar33892256
Homocysteine,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)N[C@@H](CCS)C(=O)O[Si](C)(C)C(C)(C)C1976.6Standard polar33892256
Homocysteine,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)N[C@@H](CCS[Si](C)(C)C(C)(C)C)C(=O)O2117.4Semi standard non polar33892256
Homocysteine,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)N[C@@H](CCS[Si](C)(C)C(C)(C)C)C(=O)O2052.6Standard non polar33892256
Homocysteine,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)N[C@@H](CCS[Si](C)(C)C(C)(C)C)C(=O)O2189.1Standard polar33892256
Homocysteine,2TBDMS,isomer #4CC(C)(C)[Si](C)(C)N([C@@H](CCS)C(=O)O)[Si](C)(C)C(C)(C)C2035.0Semi standard non polar33892256
Homocysteine,2TBDMS,isomer #4CC(C)(C)[Si](C)(C)N([C@@H](CCS)C(=O)O)[Si](C)(C)C(C)(C)C1949.8Standard non polar33892256
Homocysteine,2TBDMS,isomer #4CC(C)(C)[Si](C)(C)N([C@@H](CCS)C(=O)O)[Si](C)(C)C(C)(C)C2049.7Standard polar33892256
Homocysteine,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)N[C@@H](CCS[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2314.8Semi standard non polar33892256
Homocysteine,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)N[C@@H](CCS[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2315.1Standard non polar33892256
Homocysteine,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)N[C@@H](CCS[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2130.8Standard polar33892256
Homocysteine,3TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)[C@H](CCS)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2318.7Semi standard non polar33892256
Homocysteine,3TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)[C@H](CCS)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2288.1Standard non polar33892256
Homocysteine,3TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)[C@H](CCS)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2048.9Standard polar33892256
Homocysteine,3TBDMS,isomer #3CC(C)(C)[Si](C)(C)SCC[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2470.5Semi standard non polar33892256
Homocysteine,3TBDMS,isomer #3CC(C)(C)[Si](C)(C)SCC[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2370.8Standard non polar33892256
Homocysteine,3TBDMS,isomer #3CC(C)(C)[Si](C)(C)SCC[C@@H](C(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2198.3Standard polar33892256
Homocysteine,4TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@H](CCS[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2702.7Semi standard non polar33892256
Homocysteine,4TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@H](CCS[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2594.6Standard non polar33892256
Homocysteine,4TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@H](CCS[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2190.7Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - Homocysteine GC-EI-TOF (Non-derivatized)splash10-004i-0920000000-20fbffbe76510c066e6f2017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homocysteine GC-MS (Non-derivatized) - 70eV, Positivesplash10-0006-9000000000-1f800d28bca4b2b7a49f2016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homocysteine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homocysteine GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homocysteine GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homocysteine GC-MS (TMS_1_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homocysteine GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homocysteine GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Homocysteine GC-MS (TBDMS_1_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Homocysteine LC-ESI-ITFT , positive-QTOFsplash10-014l-4900000000-c50d79ba3a5013bcf29f2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homocysteine LC-ESI-ITFT , positive-QTOFsplash10-000i-9000000000-025c9e1f4fb58138c43b2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homocysteine LC-ESI-ITFT , positive-QTOFsplash10-014i-4900000000-33f205e002f49f0347642017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Homocysteine 35V, Positive-QTOFsplash10-052r-0900000000-c1f98c8182b5ddc09cb12021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 10V, Positive-QTOFsplash10-00ko-9800000000-80a1c80328e8836233a42015-09-15Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 20V, Positive-QTOFsplash10-0006-9100000000-1f611da44349ffdfaefe2015-09-15Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 40V, Positive-QTOFsplash10-0bvi-9000000000-c9d62a66522e46a17f562015-09-15Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 10V, Negative-QTOFsplash10-001i-3900000000-e8322d5858cba16a3bd22015-09-15Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 20V, Negative-QTOFsplash10-00lr-6900000000-e0190a1cc33e605bac0a2015-09-15Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 40V, Negative-QTOFsplash10-001i-9000000000-900bb5d8fb11bec02e392015-09-15Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 10V, Negative-QTOFsplash10-001i-0900000000-38e427e24202b24115972021-09-21Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 20V, Negative-QTOFsplash10-001i-7900000000-5cf2133d9faac56032d32021-09-21Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 40V, Negative-QTOFsplash10-001i-9000000000-942ac689538269d6ca7b2021-09-21Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 10V, Positive-QTOFsplash10-0006-9000000000-166428542631f9ac07142021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 20V, Positive-QTOFsplash10-0a4i-9000000000-9bcb57b924579a6c34112021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Homocysteine 40V, Positive-QTOFsplash10-0a4j-9000000000-8eee886864db64f3eec22021-09-25Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)2019-05-22Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2019-05-22Wishart 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
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Adipose Tissue
  • Brain
  • Fibroblasts
  • Intestine
  • Kidney
  • Liver
  • Neuron
  • Pancreas
  • Placenta
  • Platelet
  • Skeletal Muscle
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified9.4 (7.3-16.2) uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified9.8 (7.6-14.9) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1-18 uMInfant (0-1 year old)BothNormal
    • Clinical and Labo...
details
BloodDetected and Quantified<7.500 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified2.89-9.99 uMInfant (5 days - <1 year old)Both
Normal
    • CALIPER Paediatri...
details
BloodDetected and Quantified2.74-7.62 uMChildren (1 - <7 years old)Both
Normal
    • CALIPER Paediatri...
details
BloodDetected and Quantified3.40-8.43 uMChildren (7 - <12 years old)Both
Normal
    • CALIPER Paediatri...
details
BloodDetected and Quantified4.069-10.36 uMAdolescent (12 - <15 years old)Female
Normal
    • CALIPER Paediatri...
details
BloodDetected and Quantified4.96-11.91 uMAdolescent (15 - <19 years old)Female
Normal
    • CALIPER Paediatri...
details
BloodDetected and Quantified4.73-10.43 uMAdolescent (12 - <15 years old)Male
Normal
    • CALIPER Paediatri...
details
BloodDetected and Quantified5.47-13.39 uMAdolescent (15 - <19 years old)Male
Normal
    • CALIPER Paediatri...
details
BloodDetected and Quantified5.00 +/- 1.6 uMInfant (0-1 year old)BothNormal details
BloodDetected and Quantified10.4 +/- 2.3 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified9.00 +/- 2.2 uMAdolescent (13-18 years old)BothNormal details
BloodDetected and Quantified9.00 +/- 2.00 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified<20 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified3-8 uMNewborn (0-30 days old)BothNormal details
BloodDetected and Quantified9.99 +/- 1.04 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified8-18 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified<12.593 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified10.900 +/- 2.800 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified11.600 +/- 3 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified12.600 +/- 3.100 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified13.300 +/- 2.900 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified5.600 +/- 1.200 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified9.11 +/- 2.99 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified4-12 uMChildren (1-13 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.09 (0.06-0.16) uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified20.7 +/- 1.7 uMElderly (>65 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.057 +/- 0.035 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.12 +/- 0.08 uMElderly (>65 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.15 +/- 0.07 uMAdult (>18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
SalivaDetected and Quantified6.6 +/- 8.0 uMAdult (>18 years old)BothNormal details
SalivaDetected and Quantified2.92 +/- 2.27 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.91-1.4 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified1.1 (0.6-1.74) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified2.00 +/- 1.65 umol/mmol creatinineInfant (0-1 year old)MaleNormal details
UrineDetected and Quantified1.5 +/- 0.69 umol/mmol creatinineChildren (1-13 years old)MaleNormal details
UrineDetected and Quantified1.78 +/- 0.73 umol/mmol creatinineAdolescent (13-18 years old)MaleNormal details
UrineDetected and Quantified1.95 +/- 1.47 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.86-1.2 umol/mmol creatinineAdult (>18 years old)MaleNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified12.9 (8.2-28.2) uMAdult (>18 years old)Not SpecifiedDementia (Alzheimer's and non-Alzheimer's) details
BloodDetected and Quantified10.7 (7.6-14.4) uMAdult (>18 years old)Not SpecifiedMultiple sclerosis details
BloodDetected and Quantified10.2 (7.0-17.4) uMAdult (>18 years old)Not SpecifiedStroke details
BloodDetected and Quantified11.9 (7.0-19.1) uMAdult (>18 years old)Not SpecifiedPeripheral neuropathy details
BloodDetected and Quantified20.5 uMChildren (1-13 years old)Female
Cystathioninuria
details
BloodDetected and Quantified19 uMChildren (1-13 years old)Female
Cystathioninuria
details
BloodDetected and Quantified21.0 (18.5-25.7) uMAdult (>18 years old)BothChronic renal failure details
BloodDetected and Quantified3.5 uMChildren (1-13 years old)Female
Cystathioninuria
details
BloodDetected and Quantified25.5 (16.9-32.6) uMAdult (>18 years old)BothContinuous ambulatory peritoneal dialysis (CAPD) details
BloodDetected and Quantified29.7 (25.7-38.2) uMAdult (>18 years old)BothHemodialysis details
BloodDetected and Quantified<0.700 uMInfant (0-1 year old)Male
Sulfite oxidase deficiency
    • Clinical and Labo...
details
BloodDetected and Quantified68.80 +/- 15.53 uMAdult (>18 years old)Bothuremia details
BloodDetected and Quantified3.8 uMInfant (0-1 year old)FemalePyroglutamic aciduria details
BloodDetected and Quantified12.5 +/- 5.0 uMAdult (>18 years old)Both
Sickle cell anemia
details
BloodDetected and Quantified180 uMAdult (>18 years old)Male
Methylenetetrahydrofolate reductase deficiency
details
BloodDetected and Quantified<1.000 uMNewborn (0-30 days old)Female
Molybdenum cofactor deficiency
details
BloodDetected and Quantified12 uMAdult (>18 years old)MaleDimethylglycinuria details
BloodDetected and Quantified60 +/- 11.852 uMAdult (>18 years old)Both
Uremia
details
BloodDetected and Quantified125.00 (50.00-200.00) uMChildren (1-13 years old)BothCystathionine beta-synthase deficiency
    • MetaGene: Metabol...
details
BloodDetected and Quantified6.00 (4.8-7.40) uMAdult (>18 years old)BothHomocystinuria
    • MetaGene: Metabol...
details
BloodDetected and Quantified9.22 +/- 1.81 uMAdult (>18 years old)Not SpecifiedCruetzfeldt-Jakob disease details
BloodDetected and Quantified15.1 uMAdult (>18 years old)BothParkinson's disease details
BloodDetected and Quantified13.94 +/- 3.47 uMAdult (>18 years old)BothAmyotrophic lateral sclerosis details
BloodDetected and Quantified15.75 +/- 5.75 uMAdult (>18 years old)BothProgressive supranuclear palsy details
BloodDetected and Quantified0.9 uMChildren (1-13 years old)MaleMolybdenium co-factor deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified0.08 (0.05-0.16) uMAdult (>18 years old)Not SpecifiedMultiple sclerosis details
Cerebrospinal Fluid (CSF)Detected and Quantified0.08 (0.06-0.15) uMAdult (>18 years old)Not SpecifiedStroke details
Cerebrospinal Fluid (CSF)Detected and Quantified0.11 (0.07-0.18) uMAdult (>18 years old)Not SpecifiedPeripheral neuropathy details
Cerebrospinal Fluid (CSF)Detected and Quantified0.216 +/- 0.122 uMAdult (>18 years old)Not SpecifiedAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified0.12 +/- 0.062 uMElderly (>65 years old)Not SpecifiedAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified0.10 (0.06–0.32) uMAdult (>18 years old)Not SpecifiedDementia (Alzheimer's and non-Alzheimer's) details
Cerebrospinal Fluid (CSF)Detected and Quantified14.2 +/- 1.7 uMElderly (>65 years old)Not SpecifiedAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified0.13 +/- 0.03 uMAdult (>18 years old)Not SpecifiedCruetzfeldt-Jakob disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothOral squamous cell carcinoma (OSCC) details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothOral leukoplakia (OLK) details
UrineDetected and Quantified0.470-0.500 umol/mmol creatinineNewborn (0-30 days old)FemaleTranscobalamin II deficiency details
Associated Disorders and Diseases
Disease References
Creutzfeldt-Jakob disease
  1. Bleich S, Otto M, Zerr I, Kropp S, Kretzschmar HA, Wiltfang J: Creutzfeldt-Jakob disease and homocysteine levels in plasma and cerebrospinal fluid. Gerontology. 2005 Mar-Apr;51(2):142-4. [PubMed:15711082 ]
  2. [No authors listed] [PubMed:115711082 ]
Sickle cell anemia
  1. van der Dijs FP, Schnog JJ, Brouwer DA, Velvis HJ, van den Berg GA, Bakker AJ, Duits AJ, Muskiet FD, Muskiet FA: Elevated homocysteine levels indicate suboptimal folate status in pediatric sickle cell patients. Am J Hematol. 1998 Nov;59(3):192-8. [PubMed:9798656 ]
Multiple sclerosis
  1. Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed:17384003 ]
Stroke
  1. Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed:17384003 ]
Peripheral neuropathy
  1. Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed:17384003 ]
Chronic renal failure
  1. McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. [PubMed:11380830 ]
Continuous ambulatory peritoneal dialysis
  1. McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. [PubMed:11380830 ]
Hemodialysis
  1. McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. [PubMed:11380830 ]
Homocystinuria
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Parkinson's disease
  1. Levin J, Botzel K, Giese A, Vogeser M, Lorenzl S: Elevated levels of methylmalonate and homocysteine in Parkinson's disease, progressive supranuclear palsy and amyotrophic lateral sclerosis. Dement Geriatr Cogn Disord. 2010;29(6):553-9. doi: 10.1159/000314841. Epub 2010 Jul 3. [PubMed:20606437 ]
Amyotrophic lateral sclerosis
  1. Levin J, Botzel K, Giese A, Vogeser M, Lorenzl S: Elevated levels of methylmalonate and homocysteine in Parkinson's disease, progressive supranuclear palsy and amyotrophic lateral sclerosis. Dement Geriatr Cogn Disord. 2010;29(6):553-9. doi: 10.1159/000314841. Epub 2010 Jul 3. [PubMed:20606437 ]
Progressive supranuclear palsy
  1. Levin J, Botzel K, Giese A, Vogeser M, Lorenzl S: Elevated levels of methylmalonate and homocysteine in Parkinson's disease, progressive supranuclear palsy and amyotrophic lateral sclerosis. Dement Geriatr Cogn Disord. 2010;29(6):553-9. doi: 10.1159/000314841. Epub 2010 Jul 3. [PubMed:20606437 ]
Uremia
  1. Duranton F, Cohen G, De Smet R, Rodriguez M, Jankowski J, Vanholder R, Argiles A: Normal and pathologic concentrations of uremic toxins. J Am Soc Nephrol. 2012 Jul;23(7):1258-70. doi: 10.1681/ASN.2011121175. Epub 2012 May 24. [PubMed:22626821 ]
  2. Vanholder R, De Smet R, Glorieux G, Argiles A, Baurmeister U, Brunet P, Clark W, Cohen G, De Deyn PP, Deppisch R, Descamps-Latscha B, Henle T, Jorres A, Lemke HD, Massy ZA, Passlick-Deetjen J, Rodriguez M, Stegmayr B, Stenvinkel P, Tetta C, Wanner C, Zidek W: Review on uremic toxins: classification, concentration, and interindividual variability. Kidney Int. 2003 May;63(5):1934-43. doi: 10.1046/j.1523-1755.2003.00924.x. [PubMed:12675874 ]
Sulfite oxidase deficiency, ISOLATED
  1. Choong T. et al. (2010). Clinical and Laboratory Barriers to the Timely Diagnosis of Sulphite Oxidase Deficiency. Proceedings of Singapore Healthcare, 19(2), 94-100.. Proceedings of Singapore Healthcare.
Methylenetetrahydrofolate reductase deficiency
  1. Haworth JC, Dilling LA, Surtees RA, Seargeant LE, Lue-Shing H, Cooper BA, Rosenblatt DS: Symptomatic and asymptomatic methylenetetrahydrofolate reductase deficiency in two adult brothers. Am J Med Genet. 1993 Mar 1;45(5):572-6. [PubMed:8456826 ]
Molybdenum cofactor deficiency
  1. Sass JO, Kishikawa M, Puttinger R, Reiss J, Erwa W, Shimizu A, Sperl W: Hypohomocysteinaemia and highly increased proportion of S-sulfonated plasma transthyretin in molybdenum cofactor deficiency. J Inherit Metab Dis. 2003;26(1):80-2. [PubMed:12872846 ]
Cystathioninuria
  1. Espinos C, Garcia-Cazorla A, Martinez-Rubio D, Martinez-Martinez E, Vilaseca MA, Perez-Duenas B, Kozich V, Palau F, Artuch R: Ancient origin of the CTH alelle carrying the c.200C>T (p.T67I) variant in patients with cystathioninuria. Clin Genet. 2010 Dec;78(6):554-9. doi: 10.1111/j.1399-0004.2010.01431.x. [PubMed:20584029 ]
Molybdenium co-factor deficiency
  1. Nagappa M, Bindu PS, Taly AB, Sinha S, Bharath RD: Child Neurology: Molybdenum cofactor deficiency. Neurology. 2015 Dec 8;85(23):e175-8. doi: 10.1212/WNL.0000000000002194. [PubMed:26644055 ]
Dimethylglycine Dehydrogenase Deficiency
  1. Moolenaar SH, Poggi-Bach J, Engelke UF, Corstiaensen JM, Heerschap A, de Jong JG, Binzak BA, Vockley J, Wevers RA: Defect in dimethylglycine dehydrogenase, a new inborn error of metabolism: NMR spectroscopy study. Clin Chem. 1999 Apr;45(4):459-64. [PubMed:10102904 ]
Dementia
  1. Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed:17384003 ]
Alzheimer's disease
  1. Selley ML, Close DR, Stern SE: The effect of increased concentrations of homocysteine on the concentration of (E)-4-hydroxy-2-nonenal in the plasma and cerebrospinal fluid of patients with Alzheimer's disease. Neurobiol Aging. 2002 May-Jun;23(3):383-8. [PubMed:11959400 ]
  2. Serot JM, Barbe F, Arning E, Bottiglieri T, Franck P, Montagne P, Nicolas JP: Homocysteine and methylmalonic acid concentrations in cerebrospinal fluid: relation with age and Alzheimer's disease. J Neurol Neurosurg Psychiatry. 2005 Nov;76(11):1585-7. [PubMed:16227558 ]
  3. Smach MA, Jacob N, Golmard JL, Charfeddine B, Lammouchi T, Ben Othman L, Dridi H, Bennamou S, Limem K: Folate and homocysteine in the cerebrospinal fluid of patients with Alzheimer's disease or dementia: a case control study. Eur Neurol. 2011;65(5):270-8. doi: 10.1159/000326301. Epub 2011 Apr 8. [PubMed:21474939 ]
Colorectal cancer
  1. 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 ]
  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 ]
Transcobalamin II deficiency
  1. Bibi H, Gelman-Kohan Z, Baumgartner ER, Rosenblatt DS: Transcobalamin II deficiency with methylmalonic aciduria in three sisters. J Inherit Metab Dis. 1999 Oct;22(7):765-72. [PubMed:10518276 ]
Associated OMIM IDs
DrugBank IDDB04422
Phenol Explorer Compound IDNot Available
FooDB IDFDB001491
KNApSAcK IDC00001365
Chemspider ID82666
KEGG Compound IDC00155
BioCyc IDHOMO-CYS
BiGG IDNot Available
Wikipedia LinkHomocysteine
METLIN ID3256
PubChem Compound91552
PDB IDNot Available
ChEBI ID17588
Food Biomarker OntologyNot Available
VMH IDHCYS_L
MarkerDB IDMDB00013433
Good Scents IDNot Available
References
Synthesis ReferenceKarrenbauer, Michael; Kleemann, Axel; Luessling, Theodor; Schaefer, Fritz. D,L-Homocysteine (I). Ger. (1984), 4 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Selley ML, Close DR, Stern SE: The effect of increased concentrations of homocysteine on the concentration of (E)-4-hydroxy-2-nonenal in the plasma and cerebrospinal fluid of patients with Alzheimer's disease. Neurobiol Aging. 2002 May-Jun;23(3):383-8. [PubMed:11959400 ]
  2. Yu CK, Lakasing L, Papageorghiou AT, Spencer K, Nicolaides KH: Uterine artery Doppler and mid-trimester maternal plasma homocysteine in subsequent pre-eclampsia. J Matern Fetal Neonatal Med. 2004 Aug;16(2):134-9. [PubMed:15512726 ]
  3. Giladi N, Mordechovich M, Gruendlinger L, Shabtai H, Merims D, Naor S, Baltadzhieva R, Hausdorff JM, Gur AY, Bornstein NM: "Brain Screen": A self-referral, screening program for strokes, falls and dementia risk factors. J Neurol. 2006 Mar;253(3):307-15. Epub 2005 Oct 10. [PubMed:16208527 ]
  4. Terzolo M, Allasino B, Bosio S, Brusa E, Daffara F, Ventura M, Aroasio E, Sacchetto G, Reimondo G, Angeli A, Camaschella C: Hyperhomocysteinemia in patients with Cushing's syndrome. J Clin Endocrinol Metab. 2004 Aug;89(8):3745-51. [PubMed:15292300 ]
  5. Laxdal E, Eide GE, Amundsen SR, Dregelid EB, Pedersen G, Jonung T, Aune S: Homocysteine levels, haemostatic risk factors and restenosis after carotid thrombendarterectomy. Eur J Vasc Endovasc Surg. 2004 Sep;28(3):323-8. [PubMed:15288638 ]
  6. Akoglu B, Wondra K, Caspary WF, Faust D: Determinants of fasting total serum homocysteine levels in liver transplant recipients. Exp Clin Transplant. 2006 Jun;4(1):462-6. [PubMed:16827644 ]
  7. Krantz JS, Mack WJ, Hodis HN, Liu CR, Liu CH, Kaufman FR: Early onset of subclinical atherosclerosis in young persons with type 1 diabetes. J Pediatr. 2004 Oct;145(4):452-7. [PubMed:15480366 ]
  8. Serot JM, Barbe F, Arning E, Bottiglieri T, Franck P, Montagne P, Nicolas JP: Homocysteine and methylmalonic acid concentrations in cerebrospinal fluid: relation with age and Alzheimer's disease. J Neurol Neurosurg Psychiatry. 2005 Nov;76(11):1585-7. [PubMed:16227558 ]
  9. Hershcovici T, Schechner V, Orlin J, Harell D, Beigel Y: Effect of different LDL-apheresis methods on parameters involved in atherosclerosis. J Clin Apher. 2004;19(2):90-7. [PubMed:15274202 ]
  10. Muller T, Renger K, Kuhn W: Levodopa-associated increase of homocysteine levels and sural axonal neurodegeneration. Arch Neurol. 2004 May;61(5):657-60. [PubMed:15148140 ]
  11. Eskes TK: Homocysteine and human reproduction. Clin Exp Obstet Gynecol. 2000;27(3-4):157-67. [PubMed:11214939 ]
  12. Winkelmayer WC, Kramar R, Curhan GC, Chandraker A, Endler G, Fodinger M, Horl WH, Sunder-Plassmann G: Fasting plasma total homocysteine levels and mortality and allograft loss in kidney transplant recipients: a prospective study. J Am Soc Nephrol. 2005 Jan;16(1):255-60. Epub 2004 Nov 24. [PubMed:15563562 ]
  13. Park BH, Kim YJ, Park JS, Lee HY, Ha EH, Min JW, Park HS: [Folate and homocysteine levels during pregnancy affect DNA methylation in human placenta]. J Prev Med Public Health. 2005 Nov;38(4):437-42. [PubMed:16358830 ]
  14. Hossain GS, van Thienen JV, Werstuck GH, Zhou J, Sood SK, Dickhout JG, de Koning AB, Tang D, Wu D, Falk E, Poddar R, Jacobsen DW, Zhang K, Kaufman RJ, Austin RC: TDAG51 is induced by homocysteine, promotes detachment-mediated programmed cell death, and contributes to the cevelopment of atherosclerosis in hyperhomocysteinemia. J Biol Chem. 2003 Aug 8;278(32):30317-27. Epub 2003 May 8. [PubMed:12738777 ]
  15. Schafer SA, Mussig K, Stefan N, Haring HU, Fritsche A, Balletshofer BM: Plasma homocysteine concentrations in young individuals at increased risk of type 2 diabetes are associated with subtle differences in glomerular filtration rate but not with insulin resistance. Exp Clin Endocrinol Diabetes. 2006 Jun;114(6):306-9. [PubMed:16868889 ]
  16. Tchantchou F: Homocysteine metabolism and various consequences of folate deficiency. J Alzheimers Dis. 2006 Aug;9(4):421-7. [PubMed:16917151 ]
  17. Siroka R, Trefil L, Rajdl D, Racek J, Rusnakova H, Cibulka R, Eiselt J, Filipovsky J: Asymmetric dimethylarginine, homocysteine and renal function--is there a relation? Clin Chem Lab Med. 2005;43(10):1147-50. [PubMed:16197312 ]
  18. Onalan R, Onalan G, Gunenc Z, Karabulut E: Combining 2nd-trimester maternal serum homocysteine levels and uterine artery Doppler for prediction of preeclampsia and isolated intrauterine growth restriction. Gynecol Obstet Invest. 2006;61(3):142-8. Epub 2005 Dec 20. [PubMed:16374017 ]
  19. Robinson G, Narasimhan S, Weatherall M, Beasley R: Raised plasma homocysteine levels in alcoholism: increasing the risk of heart disease and dementia? N Z Med J. 2005 Jun 3;118(1216):U1490. [PubMed:15937525 ]
  20. Gulsen M, Yesilova Z, Bagci S, Uygun A, Ozcan A, Ercin CN, Erdil A, Sanisoglu SY, Cakir E, Ates Y, Erbil MK, Karaeren N, Dagalp K: Elevated plasma homocysteine concentrations as a predictor of steatohepatitis in patients with non-alcoholic fatty liver disease. J Gastroenterol Hepatol. 2005 Sep;20(9):1448-55. [PubMed:16105135 ]
  21. Joubert LM, Manore MM: Exercise, nutrition, and homocysteine. Int J Sport Nutr Exerc Metab. 2006 Aug;16(4):341-61. [PubMed:17136938 ]
  22. Brosnan JT: Homocysteine and cardiovascular disease: interactions between nutrition, genetics and lifestyle. Can J Appl Physiol. 2004 Dec;29(6):773-80. [PubMed:15630149 ]
  23. Duranton F, Cohen G, De Smet R, Rodriguez M, Jankowski J, Vanholder R, Argiles A: Normal and pathologic concentrations of uremic toxins. J Am Soc Nephrol. 2012 Jul;23(7):1258-70. doi: 10.1681/ASN.2011121175. Epub 2012 May 24. [PubMed:22626821 ]

Enzymes

General function:
Involved in cobalamin binding
Specific function:
Catalyzes the transfer of a methyl group from methyl-cobalamin to homocysteine, yielding enzyme-bound cob(I)alamin and methionine. Subsequently, remethylates the cofactor using methyltetrahydrofolate (By similarity).
Gene Name:
MTR
Uniprot ID:
Q99707
Molecular weight:
140525.91
Reactions
5-Methyltetrahydrofolic acid + Homocysteine → Tetrahydrofolic acid + L-Methioninedetails
General function:
Involved in zinc ion binding
Specific function:
Involved in the regulation of homocysteine metabolism. Converts betaine and homocysteine to dimethylglycine and methionine, respectively. This reaction is also required for the irreversible oxidation of choline.
Gene Name:
BHMT
Uniprot ID:
Q93088
Molecular weight:
44998.205
Reactions
Betaine + Homocysteine → Dimethylglycine + L-Methioninedetails
General function:
Involved in adenosylhomocysteinase activity
Specific function:
Not Available
Gene Name:
AHCYL2
Uniprot ID:
Q96HN2
Molecular weight:
66592.445
Reactions
S-Adenosylhomocysteine + Water → Homocysteine + Adenosinedetails
General function:
Involved in adenosylhomocysteinase activity
Specific function:
Adenosylhomocysteine is a competitive inhibitor of S-adenosyl-L-methionine-dependent methyl transferase reactions; therefore adenosylhomocysteinase may play a key role in the control of methylations via regulation of the intracellular concentration of adenosylhomocysteine.
Gene Name:
AHCY
Uniprot ID:
P23526
Molecular weight:
47715.715
Reactions
S-Adenosylhomocysteine + Water → Homocysteine + Adenosinedetails
General function:
Involved in adenosylhomocysteinase activity
Specific function:
Not Available
Gene Name:
AHCYL1
Uniprot ID:
O43865
Molecular weight:
53753.0
Reactions
S-Adenosylhomocysteine + Water → Homocysteine + Adenosinedetails
General function:
Involved in cysteine biosynthetic process from serine
Specific function:
Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury (By similarity).
Gene Name:
CBS
Uniprot ID:
P35520
Molecular weight:
60586.05
Reactions
L-Serine + Homocysteine → L-Cystathionine + Waterdetails
General function:
Involved in zinc ion binding
Specific function:
Involved in the regulation of homocysteine metabolism. Converts homocysteine to methionine using S-methylmethionine (SMM) as a methyl donor.
Gene Name:
BHMT2
Uniprot ID:
Q9H2M3
Molecular weight:
33166.69
Reactions
Betaine + Homocysteine → Dimethylglycine + L-Methioninedetails