Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis)
- 1 April 1991
- journal article
- Published by Portland Press Ltd. in Biochemical Journal
- Vol. 275 (1), 269-272
- https://doi.org/10.1042/bj2750269
Abstract
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.Keywords
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