Effects of dipropylacetate on the glycine cleavage enzyme system and glycine levels. A possible experimental approach to non-ketotic hyperglycinemia.
The effect of chronic administration of the anticonvulsive drug di-n-propylacetate (DPA) on the glycine cleavage enzyme system was studied. Glycine concentrations were monitored in blood, liver, brain and spinal cord of 10-day-old rats. DPA treatment decreases glycine cleavage activity by approximately 50% in liver, and 35% in brain. The decreased cleavage activity correlates with an increase of glycine levels in blood, liver and brain. Failure to cleave glycine characterizes a metabolic disorder known as non-ketotic hyperglycinemia, which is associated with elevated concentrations of glycine in biological fluids. The inhibitory effect of DPA may provide an experimental approach to study the biochemical and pathogenic mechanisms of non-ketotic hyperglycemia.
D000367:Age Factors; D000818:Animals; D001921:Brain / Q000378:metabolism; D005998:Glycine / Q000032:analysis / Q000378:metabolism*; D008930:Mitochondria, Liver / Q000378:metabolism; D051381:Rats; D011919:Rats, Inbred Strains; D013116:Spinal Cord / Q000032:analysis; D014635:Valproic Acid / Q000378:metabolism / Q000494:pharmacology*
D014635:Valproic Acid; D005998:Glycine