Mitochondrial Translation and Beyond: Processes Implicated in Combined Oxidative Phosphorylation Deficiencies
Open accessAbstract
Mitochondrial disorders are a heterogeneous group of often multisystemic and early fatal diseases, which are amongst the most common inherited human diseases. These disorders are caused by defects in the oxidative phosphorylation (OXPHOS) system, which comprises five multisubunit enzyme complexes encoded by both the nuclear and the mitochondrial genomes. Due to the multitude of proteins and intricacy of the processes required for a properly functioning OXPHOS system, identifying the genetic defect that underlies an OXPHOS deficiency is not an easy task, especially in the case of combined OXPHOS defects. In the present communication we give an extensive overview of the proteins and processes (in)directly involved in mitochondrial translation and the biogenesis of the OXPHOS system and their roles in combined OXPHOS deficiencies. This knowledge is important for further research into the genetic causes, with the ultimate goal to effectively prevent and cure these complex and often devastating disorders.
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Mitochondrial proteins, such as ATP synthases, are thought to be preferentially translated near the organelle.
“Mitochondrial proteins, such as ATP synthases, are thought to be preferentially translated near the organelle (Margeot et al, 2005; Smits et al, 2010; Rak et al, 2011)”
From Differential dynamics of the mammalian mRNA and protein expression response to misfolding stress (2016) · cites this paper as Smits et al, 2010
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References 255 · Cited by 202
Research works printed but not linked: 1
- [54]Cayuela A Shi Y Gustafsson CM Initial characterization of MTERF4, a paralogue of MTERF1 (mTERF) In: Proceedings of the 7th European Meeting on Mitochondrial Pathology (EUROMIT ’08) June 2008 Stockholm, Sweden 20 abstract no. 14
- Authors
- Smits, P., Smeitink, J., & van den Heuvel, L.
- Affiliations
- Radboud University Nijmegen (NL)
- Department of Pediatrics, Nijmegen Center for Mitochondrial Disorders, Radboud University Nijmegen Medical Center, Geert Grooteplein 10, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands
- Venue
- Journal of Biomedicine and Biotechnology
- Publisher
- Hindawi Limited
- Published
- 2010
- Volume
- 2010
- Pages
- 1-24
- ISSN
- 1110-7243 (ISSN-L)
- 1110-7251
- PMC
- PMC2854570
- OpenAlex
- W2005911187
- Cited by
- 202
- References
- 255
- Citations by year
- 2026: 5 · 2025: 6 · 2024: 7 · 2023: 7 · 2022: 15 · 2021: 7 · 2020: 13 · 2019: 8 · 2018: 8 · 2017: 13 · 2016: 13 · 2015: 24 · 2014: 19 · 2013: 18 · 2012: 18
- FWCI
- 1.60
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Locations
- BioMed Research International (publishedVersion, cc-by)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Radboud Repository (Radboud University) (submittedVersion, other-oa)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- Radboud Repository (Radboud University) (submittedVersion)
- Funders
- European Commission
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
- Keywords
- mitochondrial translation, mitochondrial disease, oxidative phosphorylation, mitochondrial genome, nuclear genome, genetic diagnosis, inherited mitochondrial disease
- MeSH terms
- Animals, Humans, Mitochondria, Models, Biological, Oxidative Phosphorylation, Protein Biosynthesis, Mitochondrial Proteins, Mitochondrial Diseases
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