Primary Identifier | IPR006223 | Type | Family |
Short Name | GCS_T |
description | 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 that catalyses the following reaction:(6S)-tetrahydrofolate + S-aminomethyldihydrolipoylprotein = (6R)-5,10-methylenetetrahydrofolate + NH3+ dihydrolipoylproteinThe glycine cleavage system is found in bacteria and the mitochondriaof eukaryotes. Mutations in the human T-protein gene are known to cause nonketotic hyperglycinemia []. |