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Mitochondria: Mitochondrial RNA metabolism and human disease

Nicholls, Thomas J. ; Rorbach, Joanna ; Minczuk, Michal

The international journal of biochemistry & cell biology, 2013-04, Vol.45 (4), p.845-849 [Periódico revisado por pares]

Netherlands: Elsevier Ltd

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Título:
Mitochondria: Mitochondrial RNA metabolism and human disease
Autor: Nicholls, Thomas J. ; Rorbach, Joanna ; Minczuk, Michal
Assuntos: Animals ; biochemical pathways ; Disease ; gene expression regulation ; genes ; human diseases ; Humans ; metabolism ; mitochondria ; Mitochondria - metabolism ; Mitochondria - pathology ; Mitochondrial disease ; mitochondrial genome ; mitochondrial RNA ; mutation ; proteins ; ribosomal RNA ; RNA - metabolism ; RNA processing ; RNA stability ; RNA turnover ; RNA, Mitochondrial ; transfer RNA ; tRNA modification
É parte de: The international journal of biochemistry & cell biology, 2013-04, Vol.45 (4), p.845-849
Notas: http://dx.doi.org/10.1016/j.biocel.2013.01.005
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Descrição: 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.
Editor: Netherlands: Elsevier Ltd
Idioma: Inglês

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Mitochondria: Mitochondrial RNA metabolism and human disease

Nicholls, Thomas J. ; Rorbach, Joanna ; Minczuk, Michal

Netherlands: Elsevier Ltd

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