000168593 001__ 168593
000168593 005__ 20260211123813.0
000168593 0247_ $$2doi$$a10.1016/j.jbc.2025.111070
000168593 0248_ $$2sideral$$a148005
000168593 037__ $$aART-2025-148005
000168593 041__ $$aeng
000168593 100__ $$aCunátová, Kristýna
000168593 245__ $$aThe cytochrome c oxidase subunit COX6B1 is required for redox-sensitive early assembly and late stabilization of complex IV
000168593 260__ $$c2025
000168593 5060_ $$aAccess copy available to the general public$$fUnrestricted
000168593 5203_ $$aCOX6B1 is a nuclear-encoded subunit of the human mitochondrial cytochrome c oxidase (cIV) located in its intermembrane space–facing region. The relevance of COX6B1 in mitochondrial physiopathology was highlighted by the missense pathogenic variants associated with cIV deficiency. Despite the assigned COX6B1 role as a late incorporation subunit, the COX6B1 human cell line KO exhibited a total loss of cIV. To get a deeper insight into the mechanisms driving the lack of cIV assembly or destabilization in the absence of COX6B1, we used the COX6B1 KO cell background to express alternative oxidase and COX6B1 pathogenic variants. These analyses uncovered that the COX6B1 subunit is indispensable for redox-sensitive early cIV assembly steps, besides its contribution to the stabilization of cIV in the late assembly stages. In addition, we have evidenced the incorporation of partially assembled cIV modules directly into supercomplex structures, supporting the “cooperative assembly” model for respiratory chain biogenesis.
000168593 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttps://creativecommons.org/licenses/by/4.0/deed.es
000168593 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000168593 700__ $$aVrbacký, Marek
000168593 700__ $$aKnezu, Michal
000168593 700__ $$aPecinová, Alena
000168593 700__ $$aAlán, Lukáš
000168593 700__ $$aHouštek, Josef
000168593 700__ $$0(orcid)0000-0002-2469-142X$$aFernández-Vizarra Bailey, Erika$$uUniversidad de Zaragoza
000168593 700__ $$aMrácek, Tomáš
000168593 700__ $$aPecina, Petr
000168593 7102_ $$11002$$2060$$aUniversidad de Zaragoza$$bDpto. Bioq.Biolog.Mol. Celular$$cÁrea Bioquímica y Biolog.Mole.
000168593 773__ $$g302, 2 (2025), 111070 [15 pp.]$$pJ. biol. chem.$$tJournal of Biological Chemistry$$x0021-9258
000168593 8564_ $$s6629252$$uhttps://zaguan.unizar.es/record/168593/files/texto_completo.pdf$$yVersión publicada
000168593 8564_ $$s3431298$$uhttps://zaguan.unizar.es/record/168593/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
000168593 909CO $$ooai:zaguan.unizar.es:168593$$particulos$$pdriver
000168593 951__ $$a2026-02-11-10:27:53
000168593 980__ $$aARTICLE