glycine cleavage system

glycine cleavage system
a group of four mitochondrial enzymes, designated P, H, T, and L, that together catalyze the conversion of glycine to CO2 and a one-carbon unit that can be transferred to tetrahydrofolate. Deficiency of one or more of the proteins, an autosomal recessive trait, results in nonketotic hyperglycinemia.

Medical dictionary. 2011.

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