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

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985823%3A_____%2F24%3A00598238" target="_blank" >RIV/67985823:_____/24:00598238 - isvavai.cz</a>

  • Alternative codes found

    RIV/86652036:_____/24:00598238 RIV/00216208:11310/24:10486380 RIV/00064165:_____/24:10486380 RIV/00216208:11110/24:10486380

  • Result on the web

    <a href="https://doi.org/10.1016/j.isci.2024.110560" target="_blank" >https://doi.org/10.1016/j.isci.2024.110560</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1016/j.isci.2024.110560" target="_blank" >10.1016/j.isci.2024.110560</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

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

  • Original language description

    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.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30202 - Endocrinology and metabolism (including diabetes, hormones)

Result continuities

  • Project

    Result was created during the realization of more than one project. More information in the Projects tab.

  • Continuities

    P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Others

  • Publication year

    2024

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    iScience

  • ISSN

    2589-0042

  • e-ISSN

    2589-0042

  • Volume of the periodical

    27

  • Issue of the periodical within the volume

    8

  • Country of publishing house

    US - UNITED STATES

  • Number of pages

    22

  • Pages from-to

    110560

  • UT code for WoS article

    001285662900001

  • EID of the result in the Scopus database

    2-s2.0-85199960580