PHYSIOLOGICAL SIGNIFICANCE OF GLYCINE CLEAVAGE SYSTEM IN HUMAN LIVER AS REVEALED BY STUDY OF A CASE OF HYPERGLYCINEMIA

Deficiency of the glycine cleavage activity was shown to compose the primary lesion in hyperglycinemia of nonketotic type in human infant. This led to the conclusion that the direct cleavage of glycine is quantitatively the most significant pathway of glycine catabolism in normal human individuals. Further, evidence was obtained that in human liver considerable portions of serine may be catabolized by way of the preliminary cleavage to methylene-THF and glycine followed by their further oxidation to yield CO2 as the final product. © 1969.