Recombinant Human Glycine dehydrogenase [decarboxylating], mitochondrial (GLDC), Truncated

Uniprot Id

P23378

Target Species

Human

Target Name

GLDC

Target Full Name

Glycine dehydrogenase (decarboxylating), mitochondrial

Target Function

The glycine cleavage system catalyzes the degradation of glycine. The P protein (GLDC) binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (GCSH).

Target Involvement

Non-ketotic hyperglycinemia (NKH)

Target Subcellular Location

Mitochondrion.

Target Protein Families

GcvP family

Target Synonyms

GCE; GCSP; GCSP_HUMAN; GLDC; Glycine cleavage system P protein; glycine cleavage system protein P; Glycine decarboxylase; glycine decarboxylase P protein ; Glycine dehydrogenase (decarboxylating) mitochondrial; Glycine dehydrogenase [decarboxylating]; mitochondrial; Gycine dehydrogenase (decarboxylating); HYGN1; MGC138198; MGC138200; NKH

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 P protein, which binds to glycine and enables the methylamine group from glycine to be transferred to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH).