Glycine cleavage system protein H (aminomethyl carrier)
Lipoic acid containing protein
Mitochondrial glycine cleavage system H protein
The glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein.
Defects in GCSH are a cause of non-ketotic hyperglycinemia (NKH) [MIM:605899]; also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.
Belongs to the gcvH family. Contains 1 lipoyl-binding domain.