A recent study described abnormalities in the expression of mitochondrial genes, including the upregulation of RHOU and the downregulation of the ATP5A1 subunit of complex V, the NDUFB11 subunit of complex I, and the TIMM9 protein in C9ORF72-ALS myogenic progenitors and myocytes obtained from iPSCs of C9ORF72-ALS patients [37]. Here, RHOU is linked to amyotrophic lateral sclerosis.