Key et al.11 showed that genetic ablation of mitochondrial matrix factors including the caseinolytic peptidase P (ClpP) and the transcription factor Tfam potently induced the transcription of RNF213 in various organs, suggesting that mysterin plays a role MMD-like vasculopathies via mitochondrial dysfunction as well as RNA-dependent inflammation11. Here, CLPP is linked to multiminicore myopathy.