Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis)

N A Hall; B D Lake; N N Dewji; A D Patrick

doi:10.1042/bj2750269

Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis)

Biochem J. 1991 Apr 1;275 ( Pt 1)(Pt 1):269-72. doi: 10.1042/bj2750269.

Authors

N A Hall¹, B D Lake, N N Dewji, A D Patrick

Affiliation

¹ Department of Clinical Biochemistry, Institute of Child Health, London, U.K.

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.

MeSH terms

Aged
Aging / metabolism
Blotting, Western
Brain / enzymology
Brain / ultrastructure*
Humans
Immunoenzyme Techniques
Lysosomes / enzymology*
Macromolecular Substances
Middle Aged
Mitochondria / enzymology*
Molecular Weight
Neuronal Ceroid-Lipofuscinoses / enzymology*
Proton-Translocating ATPases / metabolism*
Submitochondrial Particles / enzymology

Substances

Macromolecular Substances
Proton-Translocating ATPases