S-Sulfocysteine (T3D4434)

IdentificationTaxonomyBiological PropertiesPhysical PropertiesToxicity ProfileSpectraConcentrationsLinksReferencesGene RegulationTargets (3)XML
Targets (3)
Record Information
Version2.0
Creation Date2014-08-29 06:51:15 UTC
Update Date2014-12-24 20:26:49 UTC
Accession NumberT3D4434
Identification
Common NameS-Sulfocysteine
ClassSmall Molecule
DescriptionS-Sulfocysteine (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. (6, 7).
Compound Type
  • Amine
  • Animal Toxin
  • Food Toxin
  • Metabolite
  • Natural Compound
  • Organic Compound
Chemical Structure
Thumb
MOLSDFPDBSMILESInChI
Synonyms
Synonym
Cysteine-S-sulfate
Cysteine-S-sulfonate
Cysteine-S-sulfonic acid
Cysteinyl-S-sulfonate
Cysteinyl-S-sulfonic acid
L-Cysteine S-sulfate
S-Sulfo-L-cysteine
S-Sulphocysteine
Chemical FormulaC3H6NO5S2
Average Molecular Mass200.214 g/mol
Monoisotopic Mass199.969 g/mol
CAS Registry Number1637-71-4
IUPAC Name(2R)-2-amino-3-(sulfosulfanyl)propanoate
Traditional NameS-sulfo-L-cysteinate
SMILES[H][C@](N)(CSS(O)(=O)=O)C([O-])=O
InChI IdentifierInChI=1S/C3H7NO5S2/c4-2(3(5)6)1-10-11(7,8)9/h2H,1,4H2,(H,5,6)(H,7,8,9)/p-1/t2-/m0/s1
InChI KeyInChIKey=NOKPBJYHPHHWAN-REOHCLBHSA-M
Chemical Taxonomy
Description belongs to the class of organic compounds known as s-sulfo-l-cysteines. These are s-conjugated L-cysteine where the S-substituent is specified as a sulfo group.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentS-sulfo-L-cysteines
Alternative Parents
Substituents
  • S-sulfo-l-cysteine
  • Alpha-amino acid
  • L-alpha-amino acid
  • S-alkyl thiosulfate
  • Amino acid
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Sulfenyl compound
  • Organic oxygen compound
  • Amine
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic nitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Organopnictogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organic anion
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Biological Properties
StatusDetected and Not Quantified
OriginEndogenous
Cellular Locations
  • Cytosol
  • Extracellular
  • Membrane
  • Mitochondrion
  • Plasma Membrane
  • Synaptic Vesicle
Biofluid LocationsNot Available
Tissue Locations
  • Brain
Pathways
NameSMPDB LinkKEGG Link
Sulfur metabolismNot Availablemap00920
ApoptosisNot Availablemap04210
Glutathione biosynthesisNot AvailableNot Available
ApplicationsNot Available
Biological Roles
Chemical RolesNot Available
Physical Properties
StateSolid
AppearanceWhite powder.
Experimental Properties
PropertyValue
Melting PointNot Available
Boiling PointNot Available
SolubilityNot Available
LogPNot Available
Predicted Properties
PropertyValueSource
Water Solubility32.4 g/LALOGPS
logP-2ALOGPS
logP-2.3ChemAxon
logS-0.83ALOGPS
pKa (Strongest Acidic)-1.9ChemAxon
pKa (Strongest Basic)8.99ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area120.52 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity49.48 m³·mol⁻¹ChemAxon
Polarizability16.04 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash KeyDeposition DateView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0udi-1290000000-a605fed1bb7274e85c432016-08-01View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00di-1900000000-639e8d434476b29e7cd22016-08-01View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9200000000-a5114d144db3232e05ec2016-08-01View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0002-2900000000-37ede646876591d2cf082016-08-03View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00ds-9700000000-9e4da406118a2ae63d682016-08-03View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-00e9-9200000000-f9620c023173424ca78b2016-08-03View Spectrum
Toxicity Profile
Route of ExposureNot Available
Mechanism of ToxicityS-Sulfocysteine (SSC) is a very potent NMDA-receptor agonist. Electrophysiological studies have shown that SSC displays depolarizing properties similar to glutamate. Subcutaneous and intracerebral administration of SSC to infant rats induces brain damage. Apart from activating NMDA receptors, elevated SSC could also be neurotoxic by inhibition of the rate-limiting enzyme in glutathione synthesis (8), thereby reducing the intracellular levels of free radical scavengers. (6)
MetabolismS-Sulfocysteine is produced by reaction of inorganic sulfite and cystine by a yet unknown pathway (6). Sulfite is generated via the catabolism of sulfur-containing amino acids cysteine and methionine (1).
Toxicity ValuesNot Available
Lethal DoseNot Available
Carcinogenicity (IARC Classification)No indication of carcinogenicity to humans (not listed by IARC).
Uses/SourcesS-Sulfocysteine is produced by reaction of inorganic sulfite and cystine by a yet unknown pathway (6).
Minimum Risk LevelNot Available
Health EffectsNeurotoxicity.
SymptomsNot Available
TreatmentNot Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
DrugBank IDDB04370
HMDB IDHMDB00731
PubChem Compound ID115015
ChEMBL IDNot Available
ChemSpider IDNot Available
KEGG IDC05824
UniProt IDNot Available
OMIM ID
ChEBI ID27891
BioCyc IDNot Available
CTD IDNot Available
Stitch IDNot Available
PDB IDNot Available
ACToR IDNot Available
Wikipedia LinkNot Available
References
Synthesis Reference

Pierre M. Bore, Jean-Claude Arnaud, “Keratin polymer containing S-sulphocysteine residues, process for its preparation and the compositions containing it.” U.S. Patent US4948876, issued May, 1981.

MSDSLink
General References
  1. Belaidi AA, Schwarz G: Molybdenum cofactor deficiency: metabolic link between taurine and S-sulfocysteine. Adv Exp Med Biol. 2013;776:13-9. doi: 10.1007/978-1-4614-6093-0_2. [23392866 ]
  2. Graf WD, Oleinik OE, Jack RM, Weiss AH, Johnson JL: Ahomocysteinemia in molybdenum cofactor deficiency. Neurology. 1998 Sep;51(3):860-2. [9748040 ]
  3. Arnold GL, Greene CL, Stout JP, Goodman SI: Molybdenum cofactor deficiency. J Pediatr. 1993 Oct;123(4):595-8. [8410516 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. Moore W, Wiener HL, Meister A: Inactivation of gamma-glutamylcysteine synthetase, but not of glutamine synthetase, by S-sulfocysteine and S-sulfohomocysteine. J Biol Chem. 1987 Dec 15;262(35):16771-7. [2890640 ]
Gene Regulation
Up-Regulated GenesNot Available
Down-Regulated GenesNot Available

Targets

1. Glutamate (NMDA) receptor (Protein Group)
General Function:
Voltage-gated cation channel activity
Specific Function:
NMDA receptor subtype of glutamate-gated ion channels with high calcium permeability and voltage-dependent sensitivity to magnesium. Mediated by glycine. This protein plays a key role in synaptic plasticity, synaptogenesis, excitotoxicity, memory acquisition and learning. It mediates neuronal functions in glutamate neurotransmission. Is involved in the cell surface targeting of NMDA receptors (By similarity).
Included Proteins:
Q05586 , Q12879 , Q13224 , Q14957 , O15399 , Q8TCU5 , O60391
References
  1. 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 ]
Target Details
2. Glutamate--cysteine ligase catalytic subunit
General Function:
Magnesium ion binding
Specific Function:
Not Available
Gene Name:
GCLC
Uniprot ID:
P48506
Molecular Weight:
72765.14 Da
References
  1. Moore W, Wiener HL, Meister A: Inactivation of gamma-glutamylcysteine synthetase, but not of glutamine synthetase, by S-sulfocysteine and S-sulfohomocysteine. J Biol Chem. 1987 Dec 15;262(35):16771-7. [2890640 ]
Target Details
3. Glutamine synthetase
General Function:
Manganese ion binding
Specific Function:
This enzyme has 2 functions: it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner (By similarity). Essential for proliferation of fetal skin fibroblasts.
Gene Name:
GLUL
Uniprot ID:
P15104
Molecular Weight:
42064.15 Da
References
  1. Moore W, Wiener HL, Meister A: Inactivation of gamma-glutamylcysteine synthetase, but not of glutamine synthetase, by S-sulfocysteine and S-sulfohomocysteine. J Biol Chem. 1987 Dec 15;262(35):16771-7. [2890640 ]