Journal ArticleDOI
The assembly, regulation and function of the mitochondrial respiratory chain.
TLDR
A review of the latest assembly models for each individual complex, and also highlight the recent discoveries indicating that the formation of larger assemblies, known as respiratory supercomplexes, originates from the association of the intermediates of individual complexes is discussed in this article.Abstract:
The mitochondrial oxidative phosphorylation system is central to cellular metabolism. It comprises five enzymatic complexes and two mobile electron carriers that work in a mitochondrial respiratory chain. By coupling the oxidation of reducing equivalents coming into mitochondria to the generation and subsequent dissipation of a proton gradient across the inner mitochondrial membrane, this electron transport chain drives the production of ATP, which is then used as a primary energy carrier in virtually all cellular processes. Minimal perturbations of the respiratory chain activity are linked to diseases; therefore, it is necessary to understand how these complexes are assembled and regulated and how they function. In this Review, we outline the latest assembly models for each individual complex, and we also highlight the recent discoveries indicating that the formation of larger assemblies, known as respiratory supercomplexes, originates from the association of the intermediates of individual complexes. We then discuss how recent cryo-electron microscopy structures have been key to answering open questions on the function of the electron transport chain in mitochondrial respiration and how supercomplexes and other factors, including metabolites, can regulate the activity of the single complexes. When relevant, we discuss how these mechanisms contribute to physiology and outline their deregulation in human diseases. Mitochondrial respiration relies on five enzymatic complexes that couple electron transport with proton pumping, leading to ATP synthesis. Recent studies have shed new light on the organization, assembly and mechanisms of the respiratory complexes, including the formation of their larger assemblies — respiratory supercomplexes — and their roles in physiology.read more
Citations
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The role of mitochondrial fission in cardiovascular health and disease
TL;DR: Although mitochondrial fission is crucial for cardiac homeostasis, strong evidence indicates that dysregulation of DRP1-mediated fission contributes to the development of several cardiovascular pathologies.
Journal ArticleDOI
Mechanisms of mitochondrial respiratory adaptation
TL;DR: Mitochondrial adaptive cellular processes are important for physiological responses, including to nutrient availability, temperature and physical activity, and their failure leads to diseases associated with mitochondrial dysfunction such as metabolic and age-associated diseases and cancer.
Journal ArticleDOI
Exposure to the Tire Rubber-Derived Contaminant 6PPD-Quinone Causes Mitochondrial Dysfunction In Vitro
Hannah Mahoney,Francisco Carlos da Silva Junior,Catherine Roberts,Matthew Schultz,Xiaowen Ji,Alper James G. Alcaraz,David Montgomery,Summer Selinger,Jonathan K. Challis,John P. Giesy,L. Weber,David M. Janz,Steve Wiseman,M. Hecker,Markus Brinkmann +14 more
Journal ArticleDOI
Nrf2 as a regulator of mitochondrial function: Energy metabolism and beyond.
Noemí Esteras,Andrey Y. Abramov +1 more
TL;DR: The role of Nrf2 in the regulation of mitochondrial function and structure was reviewed in this paper , where the authors focused on NRF2 involvement in promoting mitochondrial quality control and regulation of basic aspects of mitochond function.
Journal ArticleDOI
Complexome Profiling—Exploring Mitochondrial Protein Complexes in Health and Disease
Alfredo Cabrera-Orefice,Alisa Ya. Potter,Felix Evers,Johannes F. Hevler,Sergio Guerrero-Castillo +4 more
TL;DR: The major findings obtained by complexome profiling are summarized; its advantages and current limitations are emphasized; multiple examples on how this tool could be applied to further investigate pathophysiological mechanisms are discussed and comment on the latest advances and opportunity areas to keep developing this methodology.
References
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