Recent work revealed that RQC is important for quality control of mitochondrial outer membrane-associated ribosomes [58, 70], and that inefficiency of this process can compromise mitochondrial homeostasis and contribute to the pathogenesis of Parkinson’s disease [58] and C9orf72-associated ALS/FTD [35]. This evidence concerns the gene C9orf72 and amyotrophic lateral sclerosis.