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Role of cytochrome c oxidase nuclear-encoded subunits in health and disease
- 1.0536893 - FGÚ 2021 RIV CZ eng J - Journal Article
Čunátová, Kristýna - Pajuelo-Reguera, David - Houštěk, Josef - Mráček, Tomáš - Pecina, Petr
Role of cytochrome c oxidase nuclear-encoded subunits in health and disease.
Physiological Research. Roč. 69, č. 6 (2020), s. 947-965. ISSN 0862-8408. E-ISSN 1802-9973
R&D Projects: GA ČR(CZ) GA16-13671S; GA MZd(CZ) NV19-07-00149
Institutional support: RVO:67985823
Keywords : mitochondria * OXPHOS * cytochrome c oxidase * nuclear-encoded subunits * mitochondrial diseases
OECD category: Biochemistry and molecular biology
Impact factor: 1.881, year: 2020
Method of publishing: Open access
https://www.biomed.cas.cz/physiolres/pdf/2020/69_947.pdf
Cytochrome c oxidase (COX), the terminal enzyme of mitochondrial electron transport chain, couples electron transport to oxygen with generation of proton gradient indispensable for the production of vast majority of ATP molecules in mammalian cells. The review summarizes current knowledge of COX structure and function of nuclear-encoded COX subunits, which may modulate enzyme activity according to various conditions. Moreover, some nuclear-encoded subunits possess tissue-specific and development-specific isoforms, possibly enabling fine-tuning of COX function in individual tissues. The importance of nuclearencoded subunits is emphasized by recently discovered pathogenic mutations in patients with severe mitopathies. In addition, proteins substoichiometrically associated with COX were found to contribute to COX activity regulation and stabilization of the respiratory supercomplexes. Based on the summarized data, a model of three levels of quaternary COX structure is postulated. Individual structural levels correspond to subunits of the i) catalytic center, ii) nuclear-encoded stoichiometric subunits and iii) associated proteins, which may constitute several forms of COX with varying composition and differentially regulated function.
Permanent Link: http://hdl.handle.net/11104/0314645
Number of the records: 1