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Role of cytochrome c oxidase nuclear-encoded subunits in health and disease
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SYSNO ASEP 0536893 Document Type J - Journal Article R&D Document Type Journal Article Subsidiary J Článek ve WOS Title Role of cytochrome c oxidase nuclear-encoded subunits in health and disease Author(s) Čunátová, Kristýna (FGU-C) RID, ORCID
Pajuelo-Reguera, David (FGU-C) RID, ORCID, SAI
Houštěk, Josef (FGU-C) RID, ORCID
Mráček, Tomáš (FGU-C) RID, ORCID
Pecina, Petr (FGU-C) RID, ORCIDSource Title Physiological Research. - : Fyziologický ústav AV ČR, v. v. i. - ISSN 0862-8408
Roč. 69, č. 6 (2020), s. 947-965Number of pages 19 s. Language eng - English Country CZ - Czech Republic Keywords mitochondria ; OXPHOS ; cytochrome c oxidase ; nuclear-encoded subunits ; mitochondrial diseases Subject RIV CE - Biochemistry OECD category Biochemistry and molecular biology R&D Projects GA16-13671S GA ČR - Czech Science Foundation (CSF) NV19-07-00149 GA MZd - Ministry of Health (MZ) Method of publishing Open access Institutional support FGU-C - RVO:67985823 UT WOS 000601215600002 EID SCOPUS 85098582229 DOI 10.33549/physiolres.934446 Annotation 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. Workplace Institute of Physiology Contact Lucie Trajhanová, lucie.trajhanova@fgu.cas.cz, Tel.: 241 062 400 Year of Publishing 2021 Electronic address https://www.biomed.cas.cz/physiolres/pdf/2020/69_947.pdf
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