Mitochondria: Mitochondrial RNA metabolism and human disease

作者：Nicholls Thomas J; Rorbach Joanna; Minczuk Michal^*

来源：International Journal of Biochemistry & Cell Biology, 2013, 45(4): 845-849.

DOI：10.1016/j.biocel.2013.01.005

• Mitochondrial disease
• RNA stability
• RNA processing
• RNA turnover
• tRNA modification

Post-transcriptional control of RNA stability, processing, modification, and degradation is key to the regulation of gene expression in all living cells. In mitochondria, these post-transcriptional processes are also vital for proper expression of the thirteen proteins encoded by the mitochondrial genome, as well as mitochondrial tRNAs and rRNAs. Our knowledge on mitochondrial RNA (mt-RNA) Metabolic pathways, however, is far from complete. All the proteins involved in mt-RNA metabolism are encoded by the nucleus, and must be imported into the organelle. Mutations in these nuclear genes can lead to perturbations in mitochondrial RNA processing, modification, stability and decay and thus are a cause of human mitochondrial disease. This review summarises the current knowledge on mt-RNA metabolism and its links with human mitochondrial pathologies.

• 出版日期2013-4