glycine decarboxylase; glycine dehydrogenase [decarboxylating]; glycine cleavage system P protein (GLDC)

Function

• component of the glycine cleavage system
• the P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO2 is released & the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein

Cofactor: pyridoxal phosphate

Structure

• homodimer
• belongs to the gcvP family

Compartment

mitochondria

Pathology

• defects in GLDC are a cause of non-ketotic hyperglycinemia

More general terms

• carboxy-lyase or decarboxylase
• mitochondrial protein

Component of

• glycine cleavage system

Database

• UniProt: http://www.uniprot.org/uniprot/P23378.html
• OMIM: https://mirror.omim.org/entry/238300
• OMIM: https://mirror.omim.org/entry/605899