Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels
| العنوان: | Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels |
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| المؤلفون: | Tobias Brandt, Anne Galinier, Marie Lagouge, Elisa Motori, Susanne Brodesser, Christoph Dieterich, Kjell Hultenby, Gunter Rappl, Ilian Atanassov, Nils-Göran Larsson, Arnaud Mourier |
| المصدر: | The Journal of Cell Biology J Cell Biol |
| بيانات النشر: | Rockefeller University Press, 2015. |
| سنة النشر: | 2015 |
| مصطلحات موضوعية: | Dynamins, Ubiquinone, MFN2, Respiratory chain, Oxidative phosphorylation, Biology, Mitochondrion, Mitochondrial Dynamics, Article, Oxidative Phosphorylation, GTP Phosphohydrolases, Electron Transport, Mice, chemistry.chemical_compound, Adenosine Triphosphate, Animals, MFN1, RNA, Small Interfering, Research Articles, Cells, Cultured, Mice, Knockout, Coenzyme Q10, Terpenes, fungi, Cell Biology, Mitochondria, Mice, Inbred C57BL, Biochemistry, chemistry, mitochondrial fusion, DNAJA3, RNA Interference, Energy Metabolism |
| الوصف: | Mitofusin 2 plays an unexpected role in maintaining the terpenoid biosynthesis pathway and is necessary for mitochondrial coenzyme Q biosynthesis. Mitochondria form a dynamic network within the cell as a result of balanced fusion and fission. Despite the established role of mitofusins (MFN1 and MFN2) in mitochondrial fusion, only MFN2 has been associated with metabolic and neurodegenerative diseases, which suggests that MFN2 is needed to maintain mitochondrial energy metabolism. The molecular basis for the mitochondrial dysfunction encountered in the absence of MFN2 is not understood. Here we show that loss of MFN2 leads to impaired mitochondrial respiration and reduced ATP production, and that this defective oxidative phosphorylation process unexpectedly originates from a depletion of the mitochondrial coenzyme Q pool. Our study unravels an unexpected and novel role for MFN2 in maintenance of the terpenoid biosynthesis pathway, which is necessary for mitochondrial coenzyme Q biosynthesis. The reduced respiratory chain function in cells lacking MFN2 can be partially rescued by coenzyme Q10 supplementation, which suggests a possible therapeutic strategy for patients with diseases caused by mutations in the Mfn2 gene. |
| تدمد: | 1540-8140 0021-9525 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9c8bf846891ae6736bf2b5f61f404023Test https://doi.org/10.1083/jcb.201411100Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....9c8bf846891ae6736bf2b5f61f404023 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15408140 00219525 |
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