PNPT1 and MELAS syndrome: For instance, Meseguer et al. demonstrated that mitochondrial Ago2 collaborates with Dicer to generate mt-tRNA-derived fragments and mt-tsRNAs in mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome.[56,61] Additionally, Ago2 has been proposed as a potential mediator of miRNA translocation, as it is associated with a pathway through polynucleotide phosphorylase (PNPase), facilitating the translocation of miRNA-378 into the mitochondria.[57]