Studies of the glycine cleavage enzyme system in brain from infants with glycine encephalopathy

Perry, T.L.; Urquhart, N.; Hansen, S.

Pediatric Research 11(12): 1192-1197


ISSN/ISBN: 0031-3998
PMID: 593763
DOI: 10.1203/00006450-197712000-00005
Accession: 000509129

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From a study of enzymes in the brain of 5 infants with glycine encephalopathy and 4 controls, including 2 with other types of hyperglycinaemia, it is concluded that the enzyme defect involves at least the second or H protein of the 4-protein glycine cleavage enzyme system. Signs appear only after birth. It is possible that a firmly bound endogenous inhibitor of the enzyme accumulates after birth in the brain of infants who had been protected by clearance of any such inhibitor across the placenta.