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114 In the context of ALS, the interaction of CCS with mutant SOD1 is faulty, resulting in decreased delivery of Cu to the mitochondria and the accumulation of the less-stabilized SOD1, which is prone to acquire pro-oxidants, consequently causing a toxic gain-of-function effect in motor neurons

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[DOI] [PubMed] [Google Scholar] 89.Desco M.C., Asensi M., Marquez R., Martinez-Valls J., Vento M., Pallardo F.V., Sastre J., Vina J

A aggregates enhance mitochondrial Ca 2 + uptake by modulating MCU in MPTP, resulting in mitochondrial Ca 2 + overload