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MGP001184

UniProt Annotations

Entry Information
Gene Nameglycine cleavage system protein H (aminomethyl carrier)
Protein EntryGCSH_HUMAN
UniProt IDP23434
SpeciesHuman
Comments
Comment typeDescription
CofactorName=(R)-lipoate; Xref=ChEBI:CHEBI:83088; Note=Binds 1 lipoyl cofactor covalently.;
DiseaseNon-ketotic hyperglycinemia (NKH) [MIM:605899]: Autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionThe 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).
SimilarityBelongs to the GcvH family. {ECO:0000305}.
SimilarityContains 1 lipoyl-binding domain. {ECO:0000255|PROSITE-ProRule:PRU01066, ECO:0000305}.
Subcellular LocationMitochondrion.
SubunitInteracts with GLDC (By similarity). The glycine cleavage system is composed of four proteins: P (GLDC), T (GCST), L (DLD) and H (GCSH). {ECO:0000250}.
Web ResourceName=NIEHS-SNPs; URL="http://egp.gs.washington.edu/data/gcsh/";
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