Wanders R J, Denis S, Ruiter J P, IJlst L, Dacremont G
Department of Clinical Chemistry, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands.
Biochim Biophys Acta. 1998 Jul 31;1393(1):35-40. doi: 10.1016/s0005-2760(98)00053-8.
Oxidation of straight-chain fatty acids in mitochondria involves the complicated interaction between a large variety of different enzymes. So far four different mitochondrial straight-chain acyl-CoA dehydrogenases have been identified. The physiological function of three of the four acyl-CoA dehydrogenases has been resolved in recent years especially from studies on patients suffering from certain inborn errors of mitochondrial fatty acid beta-oxidation. The physiological role of long-chain acyl-CoA dehydrogenase (LCAD) has remained obscure, however. The results described in this paper provide strong evidence suggesting that LCAD plays a central role in branched-chain fatty acid metabolism since it turns out to be the major acyl-CoA dehydrogenase reacting with 2,6-dimethylheptanoyl-CoA, a metabolite of pristanic acid, which itself is the alpha-oxidation product of phytanic acid.
线粒体中直链脂肪酸的氧化涉及多种不同酶之间复杂的相互作用。到目前为止，已鉴定出四种不同的线粒体直链酰基辅酶A脱氢酶。近年来，尤其是通过对患有某些线粒体脂肪酸β氧化先天性缺陷的患者的研究，已阐明了四种酰基辅酶A脱氢酶中三种的生理功能。然而，长链酰基辅酶A脱氢酶（LCAD）的生理作用仍不清楚。本文所述结果提供了有力证据，表明LCAD在支链脂肪酸代谢中起核心作用，因为它是与2,6-二甲基庚酰辅酶A反应的主要酰基辅酶A脱氢酶，2,6-二甲基庚酰辅酶A是降植烷酸的一种代谢产物，而降植烷酸本身是植烷酸的α氧化产物。
