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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:03:17 UTC
HMDB IDHMDB00731
Secondary Accession NumbersNone
Metabolite Identification
Common NameCysteine-S-sulfate
DescriptionCysteine-S-sulfate (SSC) is produced by reaction of inorganic sulfite and cystine by a yet unknown pathway and is a very potent NMDA-receptor agonist. Electrophysiological studies have shown that SSC displays depolarizing properties similar to glutamate. Patients affected with either Molybdenum cofactor deficiency (MOCOD, an autosomal recessive disease that leads to a combined deficiency of the enzymes sulphite oxidase, an enzyme that catalyzes the conversion of sulfite to inorganic sulfate, xanthine dehydrogenase and aldehyde oxidase) or isolated sulphite oxidase deficiency (ISOD, an extremely rare autosomal recessive disorder with identical clinical manifestations to MOCOD) excrete elevated levels of SSC. This rare disorder is associated with brain damage (seizures, spastic quadriplegia, and cerebral atrophy), mental retardation, dislocated ocular lenses, blindness, and excretion in the urine of abnormally large amounts of SSC, sulfite, and thiosulfate but no inorganic sulfate. (PMID: 17764028 , 15558695 ).
Structure
Thumb
Synonyms
ValueSource
Cysteine-S-sulfateChEBI
Cysteine-S-sulfonateChEBI
Cysteinyl-S-sulfonateChEBI
Cysteinyl-S-sulfonic acidChEBI
L-Cysteine hydrogen sulfateChEBI
L-Cysteine S-sulfateChEBI
S-SulfocysteineChEBI
S-SulphO-L-cysteineChEBI
S-SulphocysteineChEBI
Cysteine-S-sulfuric acidGenerator
Cysteine-S-sulphateGenerator
Cysteine-S-sulphuric acidGenerator
Cysteine-S-sulfonic acidGenerator
Cysteine-S-sulphonateGenerator
Cysteine-S-sulphonic acidGenerator
Cysteinyl-S-sulphonateGenerator
Cysteinyl-S-sulphonic acidGenerator
L-Cysteine hydrogen sulfuric acidGenerator
L-Cysteine hydrogen sulphateGenerator
L-Cysteine hydrogen sulphuric acidGenerator
L-Cysteine S-sulfuric acidGenerator
L-Cysteine S-sulphateGenerator
L-Cysteine S-sulphuric acidGenerator
Alaninethiosulfuric acidHMDB
L-Cysteine hydrogen sulfate (ester)HMDB
L-Cysteine hydrogen sulphate (ester)HMDB
L-Cysteine-S-sulfonateHMDB
L-Cysteinesulfonic acidHMDB
S-Cysteinesulfonic acidHMDB
Chemical FormulaC3H7NO5S2
Average Molecular Weight201.221
Monoisotopic Molecular Weight200.976563719
IUPAC Name(2R)-2-amino-3-(sulfosulfanyl)propanoic acid
Traditional NameS-sulphocysteine
CAS Registry Number1637-71-4
SMILES
N[C@@H](CSS(O)(=O)=O)C(O)=O
InChI Identifier
InChI=1S/C3H7NO5S2/c4-2(3(5)6)1-10-11(7,8)9/h2H,1,4H2,(H,5,6)(H,7,8,9)/t2-/m0/s1
InChI KeyInChIKey=NOKPBJYHPHHWAN-REOHCLBHSA-N
Chemical Taxonomy
DescriptionThis compound 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
  • S-alkyl thiosulfate
  • Sulfenyl compound
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point170 - 171 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility53.8 mg/mLALOGPS
logP-2.3ALOGPS
logP-2.3ChemAxon
logS-0.57ALOGPS
pKa (Strongest Acidic)-1.9ChemAxon
pKa (Strongest Basic)8.99ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area117.69 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity38.64 m3·mol-1ChemAxon
Polarizability16.76 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00di-0930000000-3a10270996044266f5ffView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9100000000-d25cccbc09519d08f684View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00di-9000000000-285ddd2bd242fb3f4f94View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-0udi-0090000000-67fc3cbd5242cf8812ebView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-0080-6920000000-51297f61c1e507e4fc41View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0089-9200000000-a3bb8e3eaa57447953e8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-001i-9100000000-7709278c846451c0d981View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-001i-9000000000-b8d9d78e8a4cb67d1d55View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-000i-2920000000-0b87083b8f086069245dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-00di-8910000000-8731b3ae0d4eda8996c3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-00di-9400000000-54f0f85b487cc83c6b80View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-0abc-9200000000-7c97c65eb8848afdecb2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-0udi-9500000000-a96d24f9946bb7c1a114View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-00di-0900000000-0f68d3c88020aecea493View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-0a6r-9800000000-6d5b706f037dc2097042View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-00dl-9000000000-41e2dd8cc34b88cf9a22View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-00di-4910000000-371af0521b2bd90215c3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-008j-7900000000-71cc4905c12f99250ba2View in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified48.759 +/- 24.834 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0.4 - 2.0 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified0 - 2.7 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified6.94 (0.26-22.0) umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified254.5 (171.00-338.00) uMChildren (1-13 years old)BothMolybdenum cofactor deficiency details
    BloodDetected and Quantified25 (10 - 40) uMChildren (1-13 years old)Not Specified
    Sulphite oxidase deficiency
    details
    UrineDetected and Quantified50.147 +/- 35.478 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    Associated Disorders and Diseases
    Disease References
    Sulfite oxidase deficiency
    1. Touati G, Rusthoven E, Depondt E, Dorche C, Duran M, Heron B, Rabier D, Russo M, Saudubray JM: Dietary therapy in two patients with a mild form of sulphite oxidase deficiency. Evidence for clinical and biological improvement. J Inherit Metab Dis. 2000 Feb;23(1):45-53. [10682307 ]
    2. MetaGene [Link]
    Associated OMIM IDs
    • 272300 (Sulfite oxidase deficiency)
    DrugBank IDNot Available
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB022209
    KNApSAcK IDNot Available
    Chemspider ID102939
    KEGG Compound IDC05824
    BioCyc IDNot Available
    BiGG IDNot Available
    Wikipedia LinkNot Available
    NuGOwiki LinkHMDB00731
    Metagene LinkHMDB00731
    METLIN IDNot Available
    PubChem Compound115015
    PDB IDCSU
    ChEBI ID27891
    References
    Synthesis ReferenceUbuka T; Kinuta M; Akagi R; Kiguchi S; Azumi M Reaction of S-(2-amino-2-carboxyethylsulfonyl)-L-cysteine with sulfite: synthesis of S-sulfo-L-cysteine and L-alanine 3-sulfinic acid and application to the determination of sulfite. Analytical biochemistry (1982), 126(2), 273-7.
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. Graf WD, Oleinik OE, Jack RM, Weiss AH, Johnson JL: Ahomocysteinemia in molybdenum cofactor deficiency. Neurology. 1998 Sep;51(3):860-2. [9748040 ]
    2. Arnold GL, Greene CL, Stout JP, Goodman SI: Molybdenum cofactor deficiency. J Pediatr. 1993 Oct;123(4):595-8. [8410516 ]
    3. Duran M, Aarsen G, Fokkens RH, Nibbering NM, Cats BP, de Bree PK, Wadman SK: 2-Mercaptoethanesulfonate-cysteine disulfide excretion following the administration of 2-mercaptoethanesulfonate--a pitfall in the diagnosis of sulfite oxidase deficiency. Clin Chim Acta. 1981 Mar 19;111(1):47-53. [6784974 ]
    4. Beemer FA, Duran M, Wadman SK, Cats BP: Absence of hepatic molybdenum cofactor. An inborn error of metabolism associated with lens dislocation. Ophthalmic Paediatr Genet. 1985 Apr;5(3):191-5. [3877898 ]
    5. Abbas AK, Xia W, Tranberg M, Wigstrom H, Weber SG, Sandberg M: S-sulfo-cysteine is an endogenous amino acid in neonatal rat brain but an unlikely mediator of cysteine neurotoxicity. Neurochem Res. 2008 Feb;33(2):301-7. Epub 2007 Sep 1. [17764028 ]
    6. Rashed MS, Saadallah AA, Rahbeeni Z, Eyaid W, Seidahmed MZ, Al-Shahwan S, Salih MA, Osman ME, Al-Amoudi M, Al-Ahaidib L, Jacob M: Determination of urinary S-sulphocysteine, xanthine and hypoxanthine by liquid chromatography-electrospray tandem mass spectrometry. Biomed Chromatogr. 2005 Apr;19(3):223-30. [15558695 ]