In particular, mutations in TRMU (MIM*610230), GTPBP3 (MIM*608536), and MTO1 (MIM*614667), which affect the post-transcriptional modification of the uridine located at the wobble position (U34) of certain mt-tRNAs, have been found to be associated with liver (TRMU) and heart failure (GTPBP3 and MTO1) [8–12]. The gene discussed is GTPBP3; the disease is heart failure.