Abstract
Mitochondria are one of the most important organelles in cells. Mitochondria are semi-autonomous organelles with their own genetic system, and can independently replicate, transcribe, and translate mitochondrial DNA. Translation initiation, elongation, termination, and recycling of the ribosome are four stages in the process of mitochondrial protein translation. In this process, mitochondrial protein translation factors and translation activators, mitochondrial RNA, and other regulatory factors regulate mitochondrial protein translation. Mitochondrial protein translation abnormalities are associated with a variety of diseases, including cancer, cardiovascular diseases, and nervous system diseases. Mutation or deletion of various mitochondrial protein translation factors and translation activators leads to abnormal mitochondrial protein translation. Mitochondrial tRNAs and mitochondrial ribosomal proteins are essential players during translation and mutations in genes encoding them represent a large fraction of mitochondrial diseases. Moreover, there is crosstalk between mitochondrial protein translation and cytoplasmic translation, and the imbalance between mitochondrial protein translation and cytoplasmic translation can affect some physiological and pathological processes. This review summarizes the regulation of mitochondrial protein translation factors, mitochondrial ribosomal proteins, mitochondrial tRNAs, and mitochondrial aminoacyl-tRNA synthetases (mt-aaRSs) in the mitochondrial protein translation process and its relationship with diseases. The regulation of mitochondrial protein translation and cytoplasmic translation in multiple diseases is also summarized.
Highlights
Mitochondria are important, semi-autonomous organelles in eukaryotic cells
We have shown that mammalian target of rapamycin (mTOR), which is the upstream regulator of mitochondria, can sense mitochondrial translation defects and subsequently activate cytoplasmic translation to compensate for them (Gao et al, 2016)
Mitochondrial protein translation is directly regulated by mitochondrial translation initiation, elongation, termination factors, translation activators, and mitomiRs
Summary
Mitochondria are important, semi-autonomous organelles in eukaryotic cells. Their independent genetic system includes mitochondrial DNA (mtDNA), messenger RNA (mRNA), transfer RNA (tRNA), ribosomal RNA (rRNA), and ribosomes. Two novel compound heterozygous mutations, c.944G>A, p.Cys315Tyr and c.856C>T, p.Gln286Xaa, in the TSFM gene of patients with juvenile-onset Leigh disease, ataxia, neuropathy, and optic atrophy, were reported to lead to EFTU protein degradation and marginally increased mitochondrial protein translation activity (Ahola et al, 2014). A comprehensive genomic analysis revealed that GFM1 is one gene mutation known to cause OXPHOS disease in patients with childhood-onset mitochondrial respiratory chain complex deficiencies (Kohda et al, 2016).
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
