Sulfocysteine (Plasma)

Optimal Result: 0 - 1.4 nmol/ML.

Sulfocysteine is the product of sulfite-dependent cleavage of cystine. In the pathway, cysteine becomes sulfite, which converts to sulfate via sulfite oxidase + Mo. If the pathway is blocked, sulfocysteine builds up.

Sulfocysteine is a very potent NMDA-receptor agonist. Electrophysiological studies have shown that Sulfocysteine 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 Sulfocysteine. 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 Sulfocysteine, sulfite, and thiosulfate but no inorganic sulfate.

References:

Abbas AK, Xia W, Tranberg M, Wigström 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. doi: 10.1007/s11064-007-9441-7. Epub 2007 Sep 1. PMID:17764028

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. doi: 10.1002/bmc.439. PMID:15558695

What does it mean if your Sulfocysteine (Plasma) result is too high?

Sulfocysteine is the product of sulfite-dependent cleavage of cystine. In the pathway, cysteine becomes sulfite, which converts to sulfate via sulfite oxidase + Mo. If the pathway is blocked, sulfocysteine builds up.

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