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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)