mitochondrial translation
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.
Pathological mutations in nuclear genes encoding components of this protein-synthesis machinery have been identified, suggesting that these types of mutation are a frequent cause of human genetic diseases.
References
Jacobs HT, Turnbull DM. Nuclear genes and mitochondrial translation: a new class of genetic disease. Trends Genet. 2005 Jun;21(6):312-4. PMID: #15922826#