tiglylglycine and Hypoglycemia

tiglylglycine has been researched along with Hypoglycemia* in 1 studies

Other Studies

1 other study(ies) available for tiglylglycine and Hypoglycemia

ArticleYear
Acetoacetyl CoA thiolase deficiency presenting as ketotic hypoglycemia.
    Pediatric research, 1987, Volume: 21, Issue:2

    We report two children who presented with hypoglycemia and metabolic acidosis in whom acetoacetyl-CoA thiolase (EC 2.3.1.9) measured in fibroblast homogenates was deficient. Deficiency of this enzyme is normally associated with urinary excretion of 2-methylacetoacetate and in one child the urinary excretion of 2-methylacetoacetate, 2-methyl-3-hydroxybutyrate, and tiglylglycine was raised. By contrast, in the other child, the urinary excretion of these metabolites was very low even during ketoacidosis and following an isoleucine load. We suggest that this could be due to deficiency of the extrahepatic isoenzyme, a defect that may be responsible for some of the cases of "ketotic hypoglycemia."

    Topics: Acetoacetates; Acetyl-CoA C-Acetyltransferase; Acetyltransferases; Acidosis; Female; Glycine; Humans; Hydroxybutyrates; Hypoglycemia; Infant; Ketosis

1987