Record Information
Version1.0
StatusDetected and Quantified
Creation Date2020-12-10 18:36:13 UTC
Update Date2022-08-31 06:02:40 UTC
MiMeDB IDMMDBc0000366
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
SynonymsNot Available
Chemical FormulaC3H7NO5S2
Average Molecular Weight201.221
Monoisotopic Molecular Weight200.976563719
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry NumberNot Available
SMILESNot Available
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 KeyNOKPBJYHPHHWAN-REOHCLBHSA-N