LP31555-3
Sulfocysteine
Active
Description
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, PMID: 15558695. Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.) Source: Human Metabolome Database, Sulfocysteine
Basic Part Properties
- Part Display Name
- Sulfocysteine
- Part Type
- Component (Describes the core component or analyte measured)
- Created On
- 2003-03-04
- Construct for LOINC Short Name
- Sulfocysteine
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Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| zh-CN | Chinese (China) | 磺酰半胱氨酸 Synonyms: Cys(SO3H); |
| fr-CA | French (Canada) | Sulfocystéine |
| et-EE | Estonian (Estonia) | Sulfotsüsteiin |
| es-ES | Spanish (Spain) | Sulfocisteina |
| it-IT | Italian (Italy) | Sulfocisteina |
| tr-TR | Turkish (Turkey) | Sülfosistein |
| ru-RU | Russian (Russian Federation) | Сульфоцистеин |
| nl-NL | Dutch (Netherlands) | sulfocysteïne |
| fr-BE | French (Belgium) | Sulfocystéine |
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