Arachidonic acid and other long-chain fatty acids in canine ceroid lipofuscinosis. Distribution in glycerolipids, metabolism, and pathophysiological correlations.
Arachidonic acid and other long-chain fatty acids in canine ceroid lipofuscinosis. Distribution in glycerolipids, metabolism, and pathophysiological correlations.
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花生四烯酸和其他长链脂肪酸在犬蜡状脂褐质沉积症中的作用。
DOI：
10.1007/bf02834282
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发表时间：
1985
期刊：
影响因子：
--
通讯作者：
Bazan,NG
中科院分区：
文献类型：
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作者：
Reddy,TS;Armstrong,D;Bazan,NG
患有犬类蜡质脂褐质沉积症 (CCL)† 的狗早在 5 个月大时就表现出异常脑电图，并在 24 个月大时表现出严重的紊乱或振幅非常低。蜡质颗粒随着年龄的增长而积累，并且在神经元内具有独特的特征外观，由单个单元膜包围的层状图案组成。尽管其形成的病因尚未完全阐明，但分离的颗粒富含磷脂。我们目前的研究检查了参与磷脂合成和周转的微粒体酶，并证明患有 CCL 的动物脑脂质的酰基组成与对照组相似。然而，在患病狗的大脑和小脑微粒体中，棕榈酸、亚油酸、花生四烯酸和二十二碳六烯酸活化为其辅酶A硫羟酸酯形式的活性显着低于对照组。此外，在受影响的动物中，花生四烯酸与磷脂的结合显着减少。这些结果表明花生四烯酸的代谢在蜡样脂褐质沉着症的发病机制中起重要作用。
Dogs with canine ceroid lipofuscinosis (CCL)† show an abnormal EEG as early as 5 mo of age and exhibited either severe disorganization or very low amplitudes by 24 mo. Ceroid particles accumulate with age and, within neurons, have a unique characteristic appearance consisting of lamellar patterns enclosed by a single unit membrane. Although the etiology of their formation has not been fully elucidated, isolated particles are enriched in phospholipids. Our present studies have examined microsomal enzymes involved in phospholipid synthesis and turnover and demonstrate that the acyl group composition of cerebral lipids from animals with CCL is similar to that from controls. However, the activation of palmitic, linoleic, arachidonic, and docosahexaenoic acids into their Coenzyme A thiol ester forms was significantly lower in cerebral and cerebellar microsomes of the diseased dogs than in those of the controls. In addition, the incorporation of arachidonic acid into phospholipids was significantly decreased in affected animals. These results suggest that the metabolism of arachidonic acid plays an important role in the pathogenesis of ceroid lipofuscinosis.