PMC:4573257 / 1843-2655 JSONTXT

{"project":"2_test","denotations":[{"id":"26189817-23333854-2053311","span":{"begin":811,"end":812},"obj":"23333854"},{"id":"26189817-25058219-2053311","span":{"begin":811,"end":812},"obj":"25058219"}],"text":"Mitochondria require unique and highly specialized mechanisms to maintain and express their genome (mtDNA). The mitochondrial genome encodes 13 essential subunits of the mitochondrial oxidative phosphorylation system (OXPHOS) and a set of tRNAs and rRNAs required for their translation. All protein components of the mitochondrial translation apparatus, including the mitochondrial ribosomal proteins, translation factors, aminoacyl tRNA synthetases, RNA modifying enzymes, and other auxiliary factors are encoded by nuclear genes and, after their synthesis in the cytoplasm, are delivered to mitochondria. Defective mtDNA expression, caused by mutations in either the mitochondrial or nuclear genomes, is associated with a diverse group of human disorders characterized by impaired mitochondrial respiration.1–3"}