Montgomery J A, Mamer O A, Colle E
McGill University-Medical Research Council of Canada Biomedical Mass Spectrometry Unit, Montreal, Quebec.
Biomed Environ Mass Spectrom. 1989 Jun;18(6):416-23. doi: 10.1002/bms.1200180610.
Rats given 2-octynoic acid by intraperitoneal injection excrete elevated amounts of medium-chain dicarboxylic acids and other acidic metabolites usually associated with human medium-chain acyl-CoA dehydrogenase deficiency. Onset of this organic acid profile is immediate and lasts for approximately 24 h. The induced acidosis in this animal model closely, acutely and transiently resembles the human disorder. The 2-octynoate load is also extensively omega- and psi-oxidized, and evidence is presented for the enzymic hydration of 2-octynoate to 3-ketooctanoic acid.
通过腹腔注射给予2-辛炔酸的大鼠，排泄出大量的中链二羧酸和其他酸性代谢产物，这些产物通常与人类中链酰基辅酶A脱氢酶缺乏症有关。这种有机酸谱的出现是即时的，持续约24小时。在这个动物模型中诱导的酸中毒与人类疾病密切、急性且短暂地相似。2-辛炔酸负荷也被广泛地进行ω-和ψ-氧化，并且有证据表明2-辛炔酸酶水合生成3-酮辛酸。
