The DOI matches its Crossref record; 9 of the 9 references it cites were found in a registry or an index.
Mitochondrial threshold effects
Open accessAbstract
The study of mitochondrial diseases has revealed dramatic variability in the phenotypic presentation of mitochondrial genetic defects. To attempt to understand this variability, different authors have studied energy metabolism in transmitochondrial cell lines carrying different proportions of various pathogenic mutations in their mitochondrial DNA. The same kinds of experiments have been performed on isolated mitochondria and on tissue biopsies taken from patients with mitochondrial diseases. The results have shown that, in most cases, phenotypic manifestation of the genetic defect occurs only when a threshold level is exceeded, and this phenomenon has been named the ‘phenotypic threshold effect'. Subsequently, several authors showed that it was possible to inhibit considerably the activity of a respiratory chain complex, up to a critical value, without affecting the rate of mitochondrial respiration or ATP synthesis. This phenomenon was called the ‘biochemical threshold effect'. More recently, quantitative analysis of the effects of various mutations in mitochondrial DNA on the rate of mitochondrial protein synthesis has revealed the existence of a ‘translational threshold effect'. In this review these different mitochondrial threshold effects are discussed, along with their molecular bases and the roles that they play in the presentation of mitochondrial diseases.
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Cites
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Cited by
The mitochondrial basis of aging
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Mitochondrial dysfunction in Parkinson's disease
Winklhofer, K. F., & Haass, C. (2010) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2009.08.013 · Cited by 688
Mitochondrial dynamics in adaptive and maladaptive cellular stress responses
Eisner, V., Picard, M., & Hajnóczky, G. (2018) · Nature Cell Biology · doi:10.1038/s41556-018-0133-0 · Cited by 622
Mitochondrial bioenergetics and structural network organization
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Metadata
- Authors
- ROSSIGNOL, R., FAUSTIN, B., ROCHER, C., MALGAT, M., MAZAT, J.-P., & LETELLIER, T.
- Venue
- Biochemical Journal
- Publisher
- Portland Press Ltd.
- Published
- 2003-03-15
- Volume
- 370
- Issue
- 3
- Pages
- 751-762
- ISSN
- 0264-6021 (ISSN-L)
- 1470-8728
- OpenAlex
- W2084621494
- Cited by
- 798
- References
- 9
- FWCI
- 5.90
- Type
- journal article
- Language
- en
- Open access
- bronze
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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