GCE antibody, NKH antibody, GCSH antibody, H-PROTEIN antibody, 1100001L02Rik antibody, 5730591C18Rik antibody, wu:fb14d10 antibody, wu:fw84b08 antibody, zgc:112535 antibody, BA5228 antibody, gce antibody, gcsh antibody, nkh antibody, wu:fd56h06 antibody, zgc:110557 antibody, zgc:92732 antibody, glycine cleavage system protein H antibody, glycine cleavage system protein H (aminomethyl carrier) antibody, glycine cleavage system lipoyl carrier protein GcvH antibody, glycine cleavage system H protein antibody, glycine cleavage system protein H (aminomethyl carrier), a antibody, glycine cleavage system carrier of aminomethyl moiety GcvH antibody, leucine rich repeat containing 34 antibody, glycine cleavage system protein H (aminomethyl carrier) L homeolog antibody, glycine cleavage system protein H (aminomethyl carrier), b antibody, GCSH antibody, Gcsh antibody, gcvH-2 antibody, gcvH antibody, gcsha antibody, LRRC34 antibody, gcsh.L antibody, gcshb antibody
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.,GCSH,GCE,NKH,Cancer,Signal Transduction,Endocrine & Metabolism,Amino acid metabolism,GCSH