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11 GSH depletion and the subsequent low stores of protein thiol result in both calcium release from intracellular stores and inhibition of calcium extrusion, producing a marked increase in cytosolic calcium concentration, which triggers cytotoxicity

Elevated production of ROS in C9orf72-associated ALS is also associated with abnormalities in mitochondrial function and neuroinflammation (Briehl et al., 80 DPRs interact with Atp5a1, which compromises mitochondrial functions in mice due to increased ROS production (Choi et al., 80 interacts with mitochondrial ribosomal proteins, inducing mitochondrial dysfunction, in motor neurons differentiated from C9orf72 patient iPSCs, compared to controls (Lopez-Gonzalez et al., 2016)

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