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Description
The enzyme system for cleavage of glycine (glycine cleavage system; EC 2.1.2.10), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase; MIM 238300), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme; MIM 238310), and L protein (a lipoamide dehydrogenase; MIM 238331). Glycine encephalopathy (GCE; MIM 605899), also called nonketotic hyperglycinemia (NKH), may be due to a defect in any one of these enzymes.[supplied by OMIM
Specifications
Specifications
| Antigen | GCSH |
| Applications | ELISA, Western Blot |
| Classification | Polyclonal |
| Conjugate | Unconjugated |
| Description | Mouse polyclonal antibody raised against a full-length recombinant GCSH. |
| Formulation | 50% glycerol |
| Gene | GCSH |
| Gene Accession No. | BC000790 |
| Gene Alias | GCE/NKH |
| Gene Symbols | GCSH |
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