Mitochondrial translation is the primary determinant of secondary mitochondrial complex I deficiencies.

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

doi:https://dx.doi.org/10.70200/RX202401046C

Number of the records: 1

Mitochondrial translation is the primary determinant of secondary mitochondrial complex I deficiencies.

1.

SYSNO ASEP	0605191
Document Type	A - Abstract
R&D Document Type	O - Ostatní
Title	Mitochondrial translation is the primary determinant of secondary mitochondrial complex I deficiencies.
Author(s)	Čunátová, Kristýna (FGU-C)_{RID, ORCID} Vrbacký, Marek (FGU-C)_{RID, ORCID} Puertas-Frias, Guillermo (FGU-C)_{RID, ORCID} Alán, Lukáš (FGU-C)_{RID, ORCID} Vanišová, M. (CZ) Saucedo-Rodríguez, María José (FGU-C)_RID Houštěk, Josef (FGU-C)_{RID, ORCID} Fernández-Vizarra, E. (IT) Neužil, Jiří (BTO-N)_{RID, ORCID} Pecinová, Alena (FGU-C)_{RID, ORCID, SAI} Pecina, Petr (FGU-C)_{RID, ORCID} Mráček, Tomáš (FGU-C)_{RID, ORCID}
Source Title	RedoXplore Roč. 1, č. 1 (2024)
Number of pages	1 s.
Action	International Congress :REDOX BIOLOGY: A Paradigm of the Foundation of Life /5./
Event date	27.09.2024 - 29.09.2024
VEvent location	Belgrade
Country	RS - Serbia
Event type	WRD
Language	eng - English
Country	RS - Serbia
Keywords	oxidative phosphorylation system (OXPHOS) ; ATP synthase ; biogenesis ; mitochondria
OECD category	Endocrinology and metabolism (including diabetes, hormones)
R&D Projects	LX22NPO5104 GA MŠMT - Ministry of Education, Youth and Sports (MEYS)
Institutional support	FGU-C - RVO:67985823 ; BTO-N - RVO:86652036
DOI	https://doi.org/10.70200/RX202401046C
Annotation	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.
Workplace	Institute of Physiology
Contact	Lucie Trajhanová, lucie.trajhanova@fgu.cas.cz, Tel.: 241 062 400
Year of Publishing	2025
Electronic address	https://doi.org/10.70200/RX202401046C

Number of the records: 1