FRCD

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).

(2R)-2-amino-3-sulfosulfanylpropanoic acid

NOKPBJYHPHHWAN-REOHCLBHSA-N

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

C(C(C(=O)O)N)SS(=O)(=O)O

C([C@@H](C(=O)O)N)SS(=O)(=O)O

        
            Cysteine-S-sulfate
        
            S-Sulfocysteine
        
            S-Sulphocysteine
        
            S-Sulfo-L-cysteine
        
            Cysteine-S-sulfonate
        
            Cysteinyl-S-sulfonate
        
            L-Cysteine S-sulfate
        
            Cysteinyl-S-sulfonic acid
        
            S-sulpho-L-cysteine
        
            1637-71-4
        

                

                  
                    Animal Toxin
                  

                
        

• Hydrocarbon derivatives
• Organic oxides
• Carbonyl compounds
• Organopnictogen compounds
• Monocarboxylic acids and derivatives
• Carboxylic acids
• Sulfenyl compounds
• Monoalkylamines
• Amino acids
• L-alpha-amino acids
• S-alkyl thiosulfates