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
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-09-27 07:49:11 UTC
HMDB IDHMDB0000742
Secondary Accession Numbers
  • HMDB00165
  • HMDB00742
Metabolite Identification
Common NameHomocysteine
DescriptionHomocysteine 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 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. 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 homocysteinuria, 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 ).
Structure
Thumb
Synonyms
ValueSource
2-amino-4-Mercaptobutyric acidChEBI
HcyChEBI
2-amino-4-MercaptobutyrateGenerator
(+-)-HomocysteineHMDB
(S)-2-amino-4-mercapto-ButanoateHMDB
(S)-2-amino-4-mercapto-Butanoic acidHMDB
2-amino-4-mercapto-ButanoateHMDB
2-amino-4-mercapto-Butanoic acidHMDB
2-amino-4-mercapto-Butyric acidHMDB
2-amino-4-mercapto-DL-ButyrateHMDB
2-amino-4-mercapto-DL-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
DL-Homocysteine (free base)HMDB
homo-CYSHMDB
L-2-amino-4-mercapto-Butyric acidHMDB
L-HomocysteineHMDB
Usaf b-12HMDB
Homocysteine, L isomerMeSH
Homocysteine, L-isomerMeSH
2 amino 4 Mercaptobutyric acidMeSH
L-Isomer homocysteineMeSH
Chemical FormulaC4H9NO2S
Average Molecular Weight135.185
Monoisotopic Molecular Weight135.035399227
IUPAC Name2-amino-4-sulfanylbutanoic acid
Traditional Namehomocysteine
CAS Registry Number454-29-5
SMILES
NC(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)
InChI KeyFFFHZYDWPBMWHY-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as alpha amino acids. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon).
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassCarboxylic acids and derivatives
Direct ParentAlpha amino acids
Alternative Parents
Substituents
  • Alpha-amino acid
  • Thia fatty acid
  • Fatty acyl
  • Fatty acid
  • Amino acid
  • Alkylthiol
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic oxide
  • Primary aliphatic amine
  • Organopnictogen compound
  • Carbonyl group
  • Amine
  • Organic oxygen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

  Health condition:

    Psychiatric disorders:

    Nervous system disorders:

    Gastrointestinal disorders:

    Cardiac disorders:

    Musculoskeletal and connective tissue disorders:

    Blood and lymphatic system disorders:

  Observation:

    Investigations:

Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

  Tissue and substructures:

  Organ and components:

  Cell and elements:

    Cell:

    Element:

Source:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

Role

Biological role:

Indirect biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point232 - 233 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility148 mg/mLNot Available
LogPNot AvailableNot Available
Predicted 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
Polarizability13.44 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0059-0930000000-b4e6d47b81f1810f1f31View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-004i-0910000000-7b3c5664e3b6b1930674View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-004i-0910000000-0dbe4ce28b468fb874f0View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-9730000000-952f5d413ee77e474820View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-03yi-3900000000-b5bcb65d1cfa2f5b4a83View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-0059-1950000000-09d1f6d02198624082eaView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0059-0981000000-c6d399e1bddf1f7bc057View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0059-0930000000-b4e6d47b81f1810f1f31View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-9730000000-952f5d413ee77e474820View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-03yi-3900000000-b5bcb65d1cfa2f5b4a83View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0059-1950000000-09d1f6d02198624082eaView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0006-9000000000-1f800d28bca4b2b7a49fView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00dj-9700000000-368cb861e20b107da57aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0006-9300000000-a8fd35730e901755194eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0a4l-9000000000-0e79cc37f5cb8f05cd37View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-052b-9000000000-694b412a6bed9a77958eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000f-9700000000-2e1b7e55ed5da543b557View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0006-9100000000-7e82906cac0f17bd5123View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0bvi-9000000000-7c49d870f77950b940a5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-001i-3900000000-4e1b7a42d769b9c2574cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-001i-7900000000-35b3c5fd78dec88148fbView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-001i-9000000000-533b0cacf1e578850e94View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Adipose Tissue
  • Artery
  • Brain
  • Fibroblasts
  • Intestine
  • Kidney
  • Liver
  • Muscle
  • Nerve Cells
  • Neuron
  • Pancreas
  • Placenta
  • Platelet
Pathways
NameSMPDB/PathwhizKEGG
3-Phosphoglycerate dehydrogenase deficiencyPw000698Pw000698 greyscalePw000698 simpleNot Available
Betaine MetabolismPw000012Pw000012 greyscalePw000012 simpleMap00260
Catecholamine BiosynthesisPw000017Pw000017 greyscalePw000017 simpleMap00350
Cystathionine Beta-Synthase DeficiencyPw000100Pw000100 greyscalePw000100 simpleNot Available
Dihydropyrimidine Dehydrogenase Deficiency (DHPD)Pw000198Pw000198 greyscalePw000198 simpleNot Available
Displaying entries 1 - 5 of 21 in total
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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 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 Quantified9.99 +/- 1.04 uMAdult (>18 years old)BothNormal 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
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
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 Quantified Adult (>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 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
BiofluidStatusValueAgeSexConditionReferenceDetails
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 Quantified21.0 (18.5-25.7) uMAdult (>18 years old)BothChronic renal failure 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 Quantified68.80 +/- 15.53 uMAdult (>18 years old)Bothuremia details
BloodDetected and Quantified12.5 +/- 5.0 uMAdult (>18 years old)Both
Sickle cell anemia
details
BloodDetected and Quantified125.00 (50.00-200.00) uMChildren (1-13 years old)BothCystathionine beta-synthase deficiency details
BloodDetected and Quantified6.00 (4.8-7.40) uMAdult (>18 years old)BothHomocystinuria 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
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
SalivaDetected but not Quantified Adult (>18 years old)BothOral squamous cell carcinoma (OSCC) details
SalivaDetected but not Quantified Adult (>18 years old)BothOral leukoplakia (OLK) details
Associated Disorders and Diseases
Disease References
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 ]
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 ]
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 ]
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 ]
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
    MetaGene
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 ]
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 ]
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 ]
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 ]
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 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDDBMET00508
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB001491
KNApSAcK IDC00001365
Chemspider ID757
KEGG Compound IDC00155
BioCyc IDHOMO-CYS
BiGG ID45588
Wikipedia LinkHomocysteine
METLIN ID3256
PubChem Compound778
PDB IDNot Available
ChEBI ID17230
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 ]

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
Trimethylammonioacetate + 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
Trimethylammonioacetate + Homocysteine → Dimethylglycine + L-Methioninedetails