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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-02-27 20:30:00 UTC
HMDB IDHMDB0000676
Secondary Accession Numbers
  • HMDB00676
Metabolite Identification
Common NameL-Homocystine
DescriptionHomocystine is the double-bonded form of homocysteine, but it occurs only transiently before being converted to the harmless cystathionine via a vitamin B6-dependent enzyme. Homocystine and homocysteine-cysteine mixed disulfide account for >98% of total homocysteine in plasma from healthy individuals (PMID 11592966 ). Oxidation of low-density lipoprotein (LDL) is thought to be a major factor in the pathophysiology of atherosclerosis. Elevated plasma homocysteine is an accepted risk factor for atherosclerosis, and may act through LDL oxidation, although this is controversial. However, the major thiol in plasma is cysteine, which is present at concentrations approximately 10 times greater than homocysteine; therefore homocystine in plasma is insignificant, and consequently homocystine is unlikely to influence LDL oxidation in vivo (PMID 14732479 ). Increasing evidence supports a role for an elevation of homocysteine in schizophrenia. It has been demonstrated that neural tube defects are related to a genetic defect in homocysteine metabolism. Sufficient intake of folic acid is believed to reduce this risk by enhancing methylation of homocysteine and its conversion to methionine, thereby compensating for this genetic defect (homocystinuria). Plasma homocysteine levels are elevated when folate levels are in the lower half of the normal range (PMID 16143442 ).
Structure
Data?1582752148
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

Health effect:

Disposition

Source:

Biological location:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point300 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility0.2 mg/mL at 25 °CNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility4.5 g/LALOGPS
logP-4.2ALOGPS
logP-5.3ChemAxon
logS-1.8ALOGPS
pKa (Strongest Acidic)1.8ChemAxon
pKa (Strongest Basic)9.8ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area126.64 ŲChemAxon
Rotatable Bond Count9ChemAxon
Refractivity64.38 m³·mol⁻¹ChemAxon
Polarizability26.92 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-004i-0910000000-965ac587d29ef8ef887eSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-004i-1910000000-f9e93d28a230da8e9833Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-004i-0910000000-965ac587d29ef8ef887eSpectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-004i-1910000000-f9e93d28a230da8e9833Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0fdo-9610000000-e10491cf3224336e3f4aSpectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00dl-9322000000-c71d91c4e21c8dc7e38bSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-0900000000-eed9e6a87dcd87f2429aSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-000i-9200000000-ff66e8130cc84f2f9307Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-000i-9000000000-c290f653e9d9de756e61Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0gi0-0290000000-0d5b5e0ed113894b2134Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0adi-4940000000-fe801a7a155db92879a5Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-000i-9600000000-fa16bd387fed9ef4ae35Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-0590000000-079299cfa664ba6bb1b7Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00lr-2920000000-b1d40f0689b373431812Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-01bi-9600000000-d4d8be33875dbb26d641Spectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified<2 uMChildren (1 - 13 years old)MaleNormal details
BloodDetected and Quantified4.60-12.44 uMChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified0-1 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified0.07 +/- 0.10 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified0.0 - 0.33 umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    UrineDetected and Quantified4.831 +/- 1.377 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    Abnormal Concentrations
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified39 uMChildren (1 - 13 years old)MaleHomocystinuria details
    BloodDetected and Quantified256.91 uMChildren (1-13 years old)FemaleHomocystinuria-megaloblastic anemia due to defect in cobalamin metabolism, cblG complementation type details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.0 - 0.2 uMChildren (1-13 years old)Not SpecifiedHomocystinuria details
    UrineDetected and Quantified23 umol/mmol creatinineChildren (1 - 13 years old)MaleHomocystinuria details
    UrineDetected and Quantified230 umol/mmol creatinineChildren (1 - 13 years old)Female
    Homocystinuria due to defect of N(5,10)-methylene THF deficiency
    details
    UrineDetected and Quantified18.59 +/- 15.858 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    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. (). Mordechai, Hien, and David S. Wishart. .
    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
    FoodDB 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 IDNot Available
    References
    Synthesis ReferenceNot Available
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. Tomaiuolo M, Vecchione G, Grandone E, Cocomazzi N, Casetta B, Di Minno G, Margaglione M: A new method for determination of plasma homocystine by isotope dilution and electrospray tandem mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2006 Sep 14;842(1):64-9. Epub 2006 Jun 19. [PubMed:16787765 ]
    2. Dudman NP, Guo XW, Crooks R, Xie L, Silberberg JS: Assay of plasma homocysteine: light sensitivity of the fluorescent 7-benzo-2-oxa-1, 3-diazole-4-sulfonic acid derivative, and use of appropriate calibrators. Clin Chem. 1996 Dec;42(12):2028-32. [PubMed:8969644 ]
    3. Kurczynski TW, Muir WA, Fleisher LD, Palomaki JF, Gaull GE, Rassin DK, Abramowsky C: Maternal homocystinuria: studies of an untreated mother and fetus. Arch Dis Child. 1980 Sep;55(9):721-3. [PubMed:7436540 ]
    4. Magera MJ, Lacey JM, Casetta B, Rinaldo P: Method for the determination of total homocysteine in plasma and urine by stable isotope dilution and electrospray tandem mass spectrometry. Clin Chem. 1999 Sep;45(9):1517-22. [PubMed:10471655 ]
    5. Haurani FI, Hall CA, Rubin R: Megaloblastic anemia as a result of an abnormal transcobalamin II (Cardeza). J Clin Invest. 1979 Nov;64(5):1253-9. [PubMed:500809 ]
    6. Sengupta S, Wehbe C, Majors AK, Ketterer ME, DiBello PM, Jacobsen DW: Relative roles of albumin and ceruloplasmin in the formation of homocystine, homocysteine-cysteine-mixed disulfide, and cystine in circulation. J Biol Chem. 2001 Dec 14;276(50):46896-904. Epub 2001 Oct 9. [PubMed:11592966 ]
    7. Nakano E, Williamson MP, Williams NH, Powers HJ: Copper-mediated LDL oxidation by homocysteine and related compounds depends largely on copper ligation. Biochim Biophys Acta. 2004 Jan 20;1688(1):33-42. [PubMed:14732479 ]
    8. Brown AS, Susser ES: Homocysteine and schizophrenia: from prenatal to adult life. Prog Neuropsychopharmacol Biol Psychiatry. 2005 Sep;29(7):1175-80. [PubMed:16143442 ]