TY - T1 - Editing activity for eliminating mischarged tRNAs is essential in mammalian mitochondria SN - / UR - http://hdl.handle.net/10138/231582 T3 - A1 - Hilander, Taru; Zhou, Xiao-Long; Konovalova, Svetlana; Zhang, Fu-Ping; Euro, Liliya; Shilov, Dmitri; Poutanen, Matti; Chihade, Joseph; Wang, En-Duo; Tyynismaa, Henna A2 - PB - Y1 - 2018 LA - eng AB - Accuracy of protein synthesis is enabled by the selection of amino acids for tRNA charging by aminoacyl-tRNA synthetases (ARSs), and further enhanced by the proofreading functions of some of these enzymes for eliminating tRNAs mischarged with noncognate amino acids. Mouse models of editing-defective cytoplasmic alanyl-tRNA synthetase (AlaRS) have previously demonstrated the importance of proofreading for cytoplasmic protein synthesis, with embryonic lethal and progressive neurodegeneration pheno... VO - IS - SP - OP - KW - 3111 Biomedicine; QUALITY-CONTROL; HUMAN-DISEASE; SYNTHETASE MUTATIONS; PROTEIN-SYNTHESIS; AMINOACYLATION; DEFECTS; MISTRANSLATION; RECOGNITION; POLYMERASE; PROTEASES N1 - PP - ER -