| Abstrakt: |
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 estersin vivo, inhibit mitochondrial ß-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 ß-oxidation when mitochondrial ß-oxidation is severely impaired. The use of these inhibitors provides animal models of dicarboxylic aciduria found in some inborn errors of metabolism. |
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