The DOI matches its Crossref record; 315 of the 315 references it cites were found in a registry or an index.
Structure and Organization of Mitochondrial Respiratory Complexes: A New Understanding of an Old Subject
Abstract
The enzymatic complexes of the mitochondrial respiratory chain have been extensively investigated in their structural and functional properties. A clear distinction is possible today between three complexes in which the difference in redox potential allows proton translocation (complexes I, III, and IV) and those having the mere function to convey electrons to the respiratory chain. We also have a clearer understanding of the structure and function of most respiratory complexes, of their biogenesis and regulation, and of their capacity to generate reactive oxygen species. Past investigations led to the conclusion that the complexes are randomly dispersed and functionally connected by diffusion of smaller redox components, coenzyme Q and cytochrome c. More-recent investigations by native gel electrophoresis and single-particle image processing showed the existence of supramolecular associations. Flux-control analysis demonstrated that complexes I and III in mammals and I, III, and IV in plants kinetically behave as single units, suggesting the existence of substrate channeling. This review discusses conditions affecting the formation of supercomplexes that, besides kinetic advantage, have a role in the stability and assembly of the individual complexes and in preventing excess oxygen radical formation. Disruption of supercomplex organization may lead to functional derangements responsible for pathologic changes.
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Cited by
Bonora, M., Patergnani, S., Rimessi, A., De Marchi, E., Suski, J. M., Bononi, A., Giorgi, C., Marchi, S., Missiroli, S., Poletti, F., et al. (2012) · Purinergic Signalling · doi:10.1007/s11302-012-9305-8 · Cited by 644
The role of mitochondria in energy production for human sperm motility
Piomboni, P., Focarelli, R., Stendardi, A., Ferramosca, A., & Zara, V. (2012) · International Journal of Andrology · doi:10.1111/j.1365-2605.2011.01218.x · Cited by 458
The architecture of the mammalian respirasome
Gu, J., Wu, M., Guo, R., Yan, K., Lei, J., Gao, N., & Yang, M. (2016) · Nature · doi:10.1038/nature19359 · Cited by 367
Complex I deficiency: clinical features, biochemistry and molecular genetics
Fassone, E., & Rahman, S. (2012) · Journal of Medical Genetics · doi:10.1136/jmedgenet-2012-101159 · Cited by 320
Functional role of mitochondrial respiratory supercomplexes
Genova, M. L., & Lenaz, G. (2014) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/j.bbabio.2013.11.002 · Cited by 306
Self-citation
Metadata
- Authors
- Lenaz, G., & Genova, M. L.
- Venue
- Antioxidants & Redox Signaling
- Publisher
- SAGE Publications
- Published
- 2010-04-15
- Volume
- 12
- Issue
- 8
- Pages
- 961-1008
- ISSN
- 1523-0864 (ISSN-L)
- 1557-7716
- OpenAlex
- W1998696969
- Cited by
- 260
- References
- 315
- FWCI
- 8.04
- Type
- journal article
- Language
- en
- Open access
- closed
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