Classification Term: 2178
S-sulfo-L-cysteines (ontology term: CHEMONTID:0004556)
S-conjugated L-cysteine where the S-substituent is specified as a sulfo group." []
found 1 associated metabolites at no_class-level_9 metabolite taxonomy ontology rank level.
Ancestor: L-cysteine-S-conjugates
Child Taxonomies: There is no child term of current ontology term.
Cysteine S-sulfate
C3H7NO5S2 (200.97656519999998)
Cysteine-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). Acquisition and generation of the data is financially supported in part by CREST/JST. KEIO_ID C127; [MS2] KO008902 KEIO_ID C127