TIMM22 and autosomal dominant progressive external ophthalmoplegia: A previous study modeling human mutation-associated adPEO in yeast suggested that the mutated Aac2 misfolds to place torsional stress on the mitochondrial inner membrane, leading to dysregulation of inner membrane proteins including loss of Oxa1p, a protein proposed to chaperone the insertion of proteins into the inner mitochondrial membrane, and of Tim22, which is involved in mitochondrial protein import.54