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

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985823%3A_____%2F24%3A00600275" target="_blank" >RIV/67985823:_____/24:00600275 - isvavai.cz</a>

Alternative codes found

RIV/86652036:_____/24:00600275 RIV/86652036:_____/24:00605191 RIV/67985823:_____/24:00605191

Result on the web

<a href="https://doi.org/10.1016/j.bbabio.2024.149424" target="_blank" >https://doi.org/10.1016/j.bbabio.2024.149424</a>

DOI - Digital Object Identifier

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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

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Czech description

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Type

O - Miscellaneous

CEP classification

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OECD FORD branch

30202 - Endocrinology and metabolism (including diabetes, hormones)

Project

<a href="/en/project/LX22NPO5104" target="_blank" >LX22NPO5104: National Institute for Research of Metabolic and Cardiovascular Diseases</a><br>

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2024

Confidentiality

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