Sherratt H S, Veitch R K
J Inherit Metab Dis. 1984;7 Suppl 1:52-6. doi: 10.1007/BF03047375.
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.
四种化合物，2-[5-(4-氯苯基)戊基]环氧乙烷-2-羧酸酯（POCA）、戊-4-烯酸酯、降血糖素和丙戊酸，在禁食动物中具有降血糖作用，且在体内形成异常的酰基辅酶A酯，它们通过不同机制抑制线粒体β-氧化。已知POCA、降血糖素和丙戊酸会导致二羧酸尿症。当线粒体β-氧化严重受损时，饱和二羧酸被认为是长链脂肪酸通过过氧化物酶体β-氧化产生的。使用这些抑制剂可提供在某些先天性代谢缺陷中发现的二羧酸尿症的动物模型。
