Animal models for dicarboxylic aciduria
- 1 March 1984
- journal article
- Published by Wiley in Journal of Inherited Metabolic Disease
- Vol. 7 (S1) , 52-56
- https://doi.org/10.1007/bf03047375
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 estersin vivo, inhibit mitochondrial β‐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 β‐oxidation when mitochondrial β‐oxidation is severely impaired. The use of these inhibitors provides animal models of dicarboxylic aciduria found in some inborn errors of metabolism.Keywords
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