Article
Comparative biochemical studies in fibroblasts from patients with different forms of Leigh syndrome.
Journal of inherited metabolic disease - 1 Jan 1996
Vazquez-Memije M E, Shanske S, Santorelli F M, Kranz-Eble P, Davidson E, DeVivo D C, DiMauro S
Abstract excerpt
We have compared respiratory chain enzyme activities, ATP synthesis, and ATP hydrolysis in cultured fibroblast mitochondria from patients with Leigh syndrome (LS) due to: (i) cytochrome oxidase (COX) deficiency (#6); (ii) pyruvate dehydrogenase complex (PDHC) deficiency (#4); and (iii) maternally...
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