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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-14 15:41:42 UTC
HMDB IDHMDB0000676
Secondary Accession Numbers
  • HMDB00676
Metabolite Identification
Common NameL-Homocystine
DescriptionHomocystine is the oxidized form of homocysteine. Homocystine is a dipeptide consisting of two homocysteine molecules joined by a disulfide bond. Homocysteine is a sulfur-containing amino acid that arises during methionine metabolism. Homocystine occurs only transiently before being reduced to homocysteine and converted to the harmless cystathionine via a vitamin B6-dependent enzyme. Homocystine and homocysteine-cysteine mixed disulfides account for >98% of total homocysteine in plasma from healthy individuals (PMID 11592966 ). Homocystine has been shown to stereospecifically induce endothelial nitric oxide synthase-dependent lipid peroxidation in endothelial cells, thereby inducing a vascular cell type-specific oxidative stress. This vascular stress is associated with atherothrombotic cardiovascular disease (PMID: 14980706 ). High levels of homocysteine (and homocysteine) can be found in individuals suffering from homocystinura due to cystathionine synthase deficiency (PMID: 4685596 )
Structure
Thumb
Synonyms
ValueSource
4,4'-Dithiobis(2-aminobutyric acid)HMDB
4,4'-Dithiobis-2-amino-L-butyrateHMDB
4,4'-Dithiobis-2-amino-L-butyric acidHMDB
[S-(R*,r*)]-4,4'-dithiobis-2-amino-butanoateHMDB
[S-(R*,r*)]-4,4'-dithiobis-2-amino-butanoic acidHMDB
2-Amino-4-{[(3S)-3-amino-3-carboxypropyl]disulfanyl}butanoateHMDB
2-Amino-4-{[(3S)-3-amino-3-carboxypropyl]disulphanyl}butanoateHMDB
2-Amino-4-{[(3S)-3-amino-3-carboxypropyl]disulphanyl}butanoic acidHMDB
Chemical FormulaC8H16N2O4S2
Average Molecular Weight268.354
Monoisotopic Molecular Weight268.05514839
IUPAC Name2-amino-4-{[(3S)-3-amino-3-carboxypropyl]disulfanyl}butanoic acid
Traditional Name2-amino-4-{[(3S)-3-amino-3-carboxypropyl]disulfanyl}butanoic acid
CAS Registry Number626-72-2
SMILES
NC(CCSSCC[C@H](N)C(O)=O)C(O)=O
InChI Identifier
InChI=1S/C8H16N2O4S2/c9-5(7(11)12)1-3-15-16-4-2-6(10)8(13)14/h5-6H,1-4,9-10H2,(H,11,12)(H,13,14)/t5-,6?/m0/s1
InChI KeyZTVZLYBCZNMWCF-ZBHICJROSA-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
  • Dicarboxylic acid or derivatives
  • Fatty acyl
  • Fatty acid
  • Amino acid
  • Organic disulfide
  • Dialkyldisulfide
  • Carboxylic acid
  • Sulfenyl compound
  • Organic oxygen compound
  • Organic nitrogen compound
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Hydrocarbon derivative
  • Primary aliphatic amine
  • Organic oxide
  • Organopnictogen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
Physiological effect
Disposition
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point300 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility0.2 mg/mL at 25 °CNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
Abnormal Concentrations
Associated Disorders and Diseases
Disease References
Homocystinuria-megaloblastic anemia due to defect in cobalamin metabolism, cblG complementation type
  1. Bhardwaj P, Sharma R, Sharma M: Homocystinuria: A rare condition presenting as stroke and megaloblastic anemia. J Pediatr Neurosci. 2010 Jul;5(2):129-31. doi: 10.4103/1817-1745.76110. [PubMed:21559159 ]
Homocystinuria
  1. CARSON NA, DENT CE, FIELD CM, GAULL GE: HOMOCYSTINURIA: CLINICAL AND PATHOLOGICAL REVIEW OF TEN CASES. J Pediatr. 1965 Mar;66:565-83. [PubMed:14264314 ]
  2. Kerrin D, Murdoch Eaton D, Livingston J, Henderson M, Smith M: Homocystinuria presenting with sagittal sinus thrombosis in infancy. J Child Neurol. 1996 Jan;11(1):70-1. [PubMed:8745393 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Homocystinuria due to defect of N(5,10)-methylene THF deficiency
  1. Ronge E, Kjellman B: Long term treatment with betaine in methylenetetrahydrofolate reductase deficiency. Arch Dis Child. 1996 Mar;74(3):239-41. [PubMed:8787431 ]
Associated OMIM IDs
  • 250940 (Homocystinuria-megaloblastic anemia due to defect in cobalamin metabolism, cblG complementation type)
  • 236200 (Homocystinuria)
  • 610247 (Eosinophilic esophagitis)
  • 236250 (Homocystinuria due to defect of N(5,10)-methylene THF deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022176
KNApSAcK IDNot Available
Chemspider ID62590
KEGG Compound IDC01817
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkHomocystine
METLIN IDNot Available
PubChem Compound69382
PDB IDNot Available
ChEBI ID89698
Food Biomarker OntologyNot Available
VMH IDLHCYSTIN
MarkerDB IDMDB00000215
Good Scents IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References