Hmdb loader
Read more...Show more...Show more...Show more...Show more...Show more...Show more...Show more...
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
Description
Structure
Data?1676999707
Synonyms
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
Predicted Chromatographic Properties
Spectra
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
Abnormal Concentrations
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

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