Protein Name glycine cleavage system protein H
Organism Homo sapiens
Gene ID 2653
Gene Symbol

GCSH

UniProt P23434 (GCSH_HUMAN)
Relationships Total Number of functionally related compound(s) : 41
Total Number of Articles : 33
Description

glycine cleavage system protein H

Gene Summary

Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). The protein encoded by this gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.[provided by RefSeq, Jan 2010]

synonyms
  • glycine cleavage system H protein, mitochondrial
  • glycine cleavage system protein H (aminomethyl carrier)
  • lipoic acid-containing protein
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Properties