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Experiments performed in differentiated SH-SY5Y neuroblastoma cells have shown that exposure to PS-NPs downregulates expression of the CI subunit NDUFB8, leading to diminished basal and maximal oxygen consumption rates alongside increased proton leakagefurther confirming mitochondrial CI dysfunction 32

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Excision of Expanded GAA Repeats Alleviates the Molecular Phenotype of Friedreichs Ataxia