4488 S-Sulfocysteine S-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. (A15402, A15407). 1637-71-4 115015 C3H6NO5S2 White powder. S-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 (A15403), thereby reducing the intracellular levels of free radical scavengers. (A15402) S-Sulfocysteine is produced by reaction of inorganic sulfite and cystine by a yet unknown pathway (A15402). Sulfite is generated via the catabolism of sulfur-containing amino acids cysteine and methionine (A3499). No indication of carcinogenicity to humans (not listed by IARC). S-Sulfocysteine is produced by reaction of inorganic sulfite and cystine by a yet unknown pathway (A15402). Neurotoxicity. 2014-08-29T06:51:15Z 2014-12-24T20:26:49Z C05824 27891 DB04370 true [H][C@](N)(CSS(O)(=O)=O)C([O-])=O C3H6NO5S2 InChI=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 InChIKey=NOKPBJYHPHHWAN-REOHCLBHSA-M 200.214 199.969287267 Endogenous Solid HMDB00731 <p>Pierre M. Bore, Jean-Claude Arnaud, &#8220;Keratin polymer containing S-sulphocysteine residues, process for its preparation and the compositions containing it.&#8221; U.S. Patent US4948876, issued May, 1981.</p>