Recombinant Human Glycine cleavage system H protein, mitochondrial (GCSH)

Uniprot Id

P23434

Target Species

Human

Target Name

GCSH

Target Full Name

Glycine cleavage system H protein, mitochondrial

Target Function

The glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST).

Target Involvement

Non-ketotic hyperglycinemia (NKH)

Target Subcellular Location

Mitochondrion.

Target Protein Families

GcvH family

Target Synonyms

GCSHGlycine cleavage system H protein; mitochondrial; Lipoic acid-containing protein

Target Background

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.