二十四碳烯酰辅酶A连接酶：X连锁型儿童肾上腺脑白质营养不良中脂肪酸β氧化系统的酶缺陷
Lignoceroyl-CoASH ligase: enzyme defect in fatty acid beta-oxidation system in X-linked childhood adrenoleukodystrophy.
作者信息
Hashmi M, Stanley W, Singh I
出版信息
FEBS Lett. 1986 Feb 17;196(2):247-50. doi: 10.1016/0014-5793(86)80256-3.
We have previously reported that the peroxisomal beta-oxidation system for very long chain fatty acids is defective in X-linked childhood adrenoleukodystrophy [(1984) Proc. Natl. Acad. Sci. USA 81, 4203-4207]. In order to elucidate the specific enzyme defect, we examined the oxidation of [1-14C]lignoceric acid, [1-14C]lignoceroyl-CoA and (1-14C)-labelled alpha,beta-unsaturated lignoceroyl-CoA (substrates for the 1st, 2nd, and 3rd steps of the beta-oxidation cycle, respectively). These studies suggest that the pathognomonic accumulation of very long chain fatty acids in X-linked childhood ALD may be due to the defective activity of peroxisomal very long chain (lignoceroyl-CoA) acyl-CoA ligase.
我们之前曾报道，在X连锁儿童肾上腺脑白质营养不良中，过氧化物酶体极长链脂肪酸β氧化系统存在缺陷[（1984年）《美国国家科学院院刊》81卷，4203 - 4207页]。为了阐明具体的酶缺陷，我们检测了[1 - ¹⁴C]二十四烷酸、[1 - ¹⁴C]二十四烷酰辅酶A和（1 - ¹⁴C）标记的α,β - 不饱和二十四烷酰辅酶A（分别为β氧化循环第一步、第二步和第三步的底物）的氧化情况。这些研究表明，X连锁儿童肾上腺脑白质营养不良中极长链脂肪酸的特征性蓄积可能是由于过氧化物酶体极长链（二十四烷酰辅酶A）酰基辅酶A连接酶活性缺陷所致。
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