Yoshida Y, Singh I
Department of Pediatrics, Medical University of South Carolina, Charleston 29425.
Biochem Med Metab Biol. 1990 Feb;43(1):22-9. doi: 10.1016/0885-4505(90)90004-k.
The effect of a 2-week clofibrate (0.5%)-fortified diet on peroxisomal palmitoyl-CoA and lignoceroyl-CoA ligases was studied. The activities of palmitoyl-CoA and lignoceroyl-CoA ligases in peroxisomes isolated from clofibrate-treated animals were 4.4- and 4.0-fold higher than those of the controls. The different degrees of increases in these two enzyme activities support the previous conclusions that in peroxisomes palmitoyl-CoA ligase and lignoceroyl-CoA ligase are different enzymes. Since clofibrate treatment increases both of these peroxisomal acyl-CoA ligase activities and normal palmitoyl-CoA ligase is the source of the partial activity for the oxidation of lignoceric acid in X-ALD, treatment with a hypolipidemic drug, which can increase human peroxisomal enzyme activities, may be helpful in lowering the amount of the pathogen, VLC fatty acids, in X-ALD.
研究了为期2周的含氯贝丁酯（0.5%）强化饮食对过氧化物酶体棕榈酰辅酶A和二十四烷酰辅酶A连接酶的影响。从经氯贝丁酯处理的动物中分离出的过氧化物酶体中，棕榈酰辅酶A和二十四烷酰辅酶A连接酶的活性分别比对照组高4.4倍和4.0倍。这两种酶活性的不同程度增加支持了先前的结论，即在过氧化物酶体中，棕榈酰辅酶A连接酶和二十四烷酰辅酶A连接酶是不同的酶。由于氯贝丁酯处理会增加这两种过氧化物酶体酰基辅酶A连接酶的活性，且正常的棕榈酰辅酶A连接酶是X-连锁肾上腺脑白质营养不良中二十四烷酸氧化部分活性的来源，因此使用一种能增加人类过氧化物酶体酶活性的降血脂药物进行治疗，可能有助于降低X-连锁肾上腺脑白质营养不良中病原体极长链脂肪酸的含量。
