Specific delay in the degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis is derived from cellular proteolytic dysfunction rather than structural alteration of subunit c

Journal of Neurochemistry

Volumn 67, Issue 4, 1996, Pages 1677-1687

  Ezaki, Junji ^a   Wolfe, Leonhard S ^b   Kominami, Eiki ^a  

^a JUNTENDO UNIVERSITY   (Japan)

^b MCGILL UNIVERSITY   (Canada)

Author keywords

Batten's disease; Lysosomes; Mitochondrial ATP synthase; Proteolysis; Subunit c

Indexed keywords

OUTER MEMBRANE PROTEIN; PEPSTATIN; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE; SUPEROXIDE DISMUTASE;

ARTICLE; ENZYME STRUCTURE; ENZYME SUBUNIT; FIBROBLAST; HUMAN; HUMAN TISSUE; LYSOSOME; LYSOSOME STORAGE DISEASE; NERVE DEGENERATION; NEURONAL CEROID LIPOFUSCINOSIS; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN PROCESSING; PROTEIN QUATERNARY STRUCTURE; STRUCTURE ANALYSIS;

EID: 0029817316     PISSN: 00223042     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1471-4159.1996.67041677.x     Document Type: Article

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