Notably, microRNA-335/335* regulates GTPBP3 and MTO1 expression.738 High ROS levels induce microRNA-9/9* expression via the NF-κB pathway, directly targeting and reducing TRMU, GTPBP3, and MTO1 mRNA and protein levels, impairing non-mutant mt-tRNA modifications and worsening the MELAS phenotype.264 Consequently, microRNA antagonists may offer potential strategies to counteract these deleterious effects.264,738. Here, GTPBP3 is linked to MELAS.