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Description

Liu B, Wang Y, Chen Y, et al

Hepatomegaly Prognosis: Death in infancy or childhood Lab Serum: Lactic Acidosis Tissue biochemistry Selective reduction in oligomycin sensitive ATPase activity (18 to 33% of normal) Short half-life of ATPase subunit Reduced Proton leak in inner mitochondrial membrane Complex V (ATP synthase) deficiency (MC5DN1) 39 ATP synthase F1 complex assembly factor-2 (ATPAF2

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