title: Animal models for dicarboxylic aciduria.
abstract: Four compounds, 2[5(4-chlorophenyl)pentyl] oxirane-2-carboxylate (POCA), pent-4-enoate, hypoglycin and valproate, which are hypoglycaemic in fasted animals and form unusual acyl-CoA esters in vivo, inhibit mitochondrial beta-oxidation by different mechanisms. POCA, hypoglycin and valproate are known to cause dicarboxylic aciduria. Saturated dicarboxylic acids are thought to be derived from long chain fatty acids by peroxisomal beta-oxidation when mitochondrial beta-oxidation is severely impaired. The use of these inhibitors provides animal models of dicarboxylic aciduria found in some inborn errors of metabolism.
mesh_terms: D000818:Animals; D003998:Dicarboxylic Acids / Q000652:urine*; D004195:Disease Models, Animal; D004852:Epoxy Compounds / Q000494:pharmacology; D005229:Fatty Acids, Monounsaturated; D005231:Fatty Acids, Unsaturated / Q000494:pharmacology; D007005:Hypoglycins / Q000494:pharmacology; D008661:Metabolism, Inborn Errors / Q000209:etiology*; D008928:Mitochondria / Q000187:drug effects / Q000378:metabolism; D012257:Riboflavin Deficiency / Q000209:etiology; D014635:Valproic Acid / Q000494:pharmacology
chemical_list: D003998:Dicarboxylic Acids; D004852:Epoxy Compounds; D005229:Fatty Acids, Monounsaturated; D005231:Fatty Acids, Unsaturated; D007005:Hypoglycins; D014635:Valproic Acid; C033891:ethyl 2-(5-(4-chlorophenyl)pentyl)oxiran-2-carboxylate; C009785:4-pentenoic acid