0605191 - FGÚ 2025 RIV RS eng A - Abstrakt
Čunátová, Kristýna - Vrbacký, Marek - Puertas-Frias, Guillermo - Alán, Lukáš - Vanišová, M. - Saucedo-Rodríguez, María José - Houštěk, Josef - Fernández-Vizarra, E. - Neužil, Jiří - Pecinová, Alena - Pecina, Petr - Mráček, Tomáš
Mitochondrial translation is the primary determinant of secondary mitochondrial complex I deficiencies.
RedoXplore. Roč. 1, č. 1 (2024). E-ISSN 3042-1772.
[International Congress :REDOX BIOLOGY: A Paradigm of the Foundation of Life /5./. 27.09.2024-29.09.2024, Belgrade]
Grant CEP: GA MŠMT(CZ) LX22NPO5104
Institucionální podpora: RVO:67985823 ; RVO:86652036
Klíčová slova: oxidative phosphorylation system (OXPHOS) * ATP synthase * biogenesis * mitochondria
Obor OECD: Endocrinology and metabolism (including diabetes, hormones)
Web výsledku:
https://doi.org/10.70200/RX202401046CDOI: https://doi.org/10.70200/RX202401046C

Individual complexes of the mitochondrial oxidative phosphorylation system (OXPHOS) are not linked solely by their function, they also share dependencies at the maintenance/assembly level, where one complex depends on the presence of a different individual complex. Despite the relevance of this “interdependence” behavior for mitochondrial diseases, its true nature remains elusive. To understand the mechanism that can explain this phenomenon, we examined the consequences of the aberration of different OXPHOS complexes in human cells. We demonstrate here that the complete disruption of each of the OXPHOS complexes resulted in a decrease in the complex I (cI) level and that the major reason for this is linked to the downregulation of mitochondrial ribosomal proteins. We conclude that the secondary cI defect is due to mitochondrial protein synthesis attenuation, while the responsible signaling pathways could differ based on the origin of the OXPHOS defect.
Trvalý link: https://hdl.handle.net/11104/0362691