InterPro domain: IPR006223

General Information

  • Identifier IPR006223
  • Description Glycine cleavage system T protein
  • Number of genes 134
  • Gene duplication stats Loading...
  • Associated GO terms GO:0004047   GO:0006546  

Abstract

The glycine cleavage system (GCS) is a multienzyme system composed of proteins P, H, T, and L, that catalyses the reversible oxidation of glycine. The T protein is an aminomethyl transferase 2.1.2.10 that catalyses the following reaction: (6S)-tetrahydrofolate + S-aminomethyldihydrolipoylprotein = (6R)-5,10-methylenetetrahydrofolate + NH 3 + dihydrolipoylprotein

The glycine cleavage system is found in bacteria and the mitochondriaof eukaryotes. Mutations in the human T-protein gene are known to cause nonketotic hyperglycinemia [ 1 ].


1. Crystal structure of aminomethyltransferase in complex with dihydrolipoyl-H-protein of the glycine cleavage system: implications for recognition of lipoyl protein substrate, disease-related mutations, and reaction mechanism. J. Biol. Chem. 285, 18684-92

Species distribution

Gene table