Accumulation of mitochondrial ATP synthase subunit c in muscle in a patient with neuronal ceroid lipofuscinosis (late infantile form)

1997 ◽  
Vol 93 (6) ◽  
pp. 628-632 ◽  
Author(s):  
F. Umehara ◽  
Itsuro Higuchi ◽  
Kenji Tanaka ◽  
Takahito Niiyama ◽  
Junji Ezaki ◽  
...  
Keyword(s):  
Atp Synthase ◽  
Neuronal Ceroid Lipofuscinosis ◽  
Infantile Form ◽  
Subunit C ◽  
Ceroid Lipofuscinosis ◽  
Mitochondrial Atp Synthase

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

1991 ◽  
Vol 275 (1) ◽  
pp. 269-272 ◽  
Author(s):  
N A Hall ◽  
B D Lake ◽  
N N Dewji ◽  
A D Patrick
Keyword(s):  
Atp Synthase ◽  
Lysosomal Storage ◽  
Infantile Form ◽  
Subunit C ◽  
Ceroid Lipofuscinosis ◽  
Mitochondrial Atp Synthase ◽  
Batten's Disease ◽  
Defective Metabolism

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.

scholarly journals Lysine methylation of mitochondrial ATP synthase subunit c stored in tissues of dogs with hereditary ceroid lipofuscinosis

1994 ◽  
Vol 269 (13) ◽  
pp. 9906-9911
Author(s):  
M.L. Katz ◽  
J.S. Christianson ◽  
N.E. Norbury ◽  
C.L. Gao ◽  
A.N. Siakotos ◽  
...  
Keyword(s):  
Atp Synthase ◽  
Lysine Methylation ◽  
Subunit C ◽  
Ceroid Lipofuscinosis ◽  
Mitochondrial Atp Synthase
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