Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis).
Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis).
复制标题
巴顿氏病（蜡质脂褐质沉积症）中线粒体 ATP 合酶 c 亚基的溶酶体储存。
作者：
N. Hall;B. Lake;N. Dewji;A. D. Patrick
免疫化学研究表明，线粒体ATP合酶的亚基c储存在晚期婴儿，青少年和成人形式的巴滕氏病。它不会以婴儿形式或在涉及溶酶体肥大的其他情况下积聚。这些结果表明，亚基c的代谢缺陷是这三种形式的巴滕氏病的发病机制的核心。
Immunochemical studies demonstrate that subunit c of mitochondrial ATP synthase is stored in the late-infantile, juvenile and adult forms of Batten's disease. It does not accumulate in the infantile form, or in other conditions involving lysosomal hypertrophy. These results suggest that the defective metabolism of subunit c is central to the pathogenesis of these three forms of Batten's disease.