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 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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Sherratt, H.S.A., Veitch, R.K. (1984). Animal Models for Dicarboxylic Aciduria. In: Addison, G.M., Chalmers, R.A., Divry, P., Harkness, R.A., Pollitt, R.J. (eds) Organic Acidurias. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-5612-4_12
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DOI: https://doi.org/10.1007/978-94-009-5612-4_12
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