Journal
TRENDS IN GENETICS
Volume 21, Issue 6, Pages 312-314Publisher
ELSEVIER SCIENCE LONDON
DOI: 10.1016/j.tig.2005.04.003
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Funding
- Wellcome Trust [074454] Funding Source: Medline
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Mitochondria contain a separate protein-synthesis machinery to produce the polypeptides encoded in mitochondrial DNA (mtDNA), and many mtDNA disease mutations affect this machinery. In humans, the mitochondrial rRNAs and tRNAs are encoded by mtDNA, whereas all proteins involved in mitochondrial translation are encoded by nuclear genes. Recently, several articles have discussed the identification of pathological mutations in nuclear genes encoding components of this protein-synthesis machinery, suggesting that these types of mutation are a frequent cause of human genetic diseases.
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