The mutations that underlie the mitochondriopathies Myoclonic Epilepsy with Ragged Red Fibers (MERRF) and some variants of Mitochondrial Encephalomyopathy, Lactic Acidosis, and Strokelike Episodes (MELAS) create
defective versions of particular mitochondrial amino acid tRNAs, leading to inefficient translation of
electron transport system (ETS) subunit proteins.