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Defective mitochondrial COX1 translation due to loss of COX14 function triggers ROS-induced inflammation in mouse liver

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Kaufmann,  S.
Research Group of Bioanalytical Mass Spectrometry, Max Planck Institute for Multidisciplinary Sciences, Max Planck Society;

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Silbern,  I.
Research Group of Bioanalytical Mass Spectrometry, Max Planck Institute for Multidisciplinary Sciences, Max Planck Society;

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Riedel,  D.
Facility for Transmission Electron Microscopy Fassberg Campus, Max Planck Institute for Multidisciplinary Sciences, Max Planck Society;

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Michanski,  S.
Department of Neurogenetics, Max Planck Institute for Multidisciplinary Sciences, Max Planck Society;

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Möbius,  W.
Department of Neurogenetics, Max Planck Institute for Multidisciplinary Sciences, Max Planck Society;

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Urlaub,  H.
Research Group of Bioanalytical Mass Spectrometry, Max Planck Institute for Multidisciplinary Sciences, Max Planck Society;

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Jakobs,  S.       
Department of NanoBiophotonics, Max Planck Institute for Multidisciplinary Sciences, Max Planck Society;
Research Group of Mitochondrial Structure and Dynamics, Max Planck Institute for Multidisciplinary Sciences, Max Planck Society;

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Rehling,  P.
MPI-NAT Fellow Mitochondrial Biogenesis and Assembly of membrane Protein Complexes, Max Planck Institute for Multidisciplinary Sciences, Max Planck Society;

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Citation

Aich, A., Boshnakovska, A., Witte, S., Gall, T., Unthan-Fechner, K., Yousefi, R., et al. (2024). Defective mitochondrial COX1 translation due to loss of COX14 function triggers ROS-induced inflammation in mouse liver. Nature Communications, 15: 6914. doi:10.1038/s41467-024-51109-y.


Cite as: https://hdl.handle.net/21.11116/0000-000F-C608-9
Abstract
Mitochondrial oxidative phosphorylation (OXPHOS) fuels cellular ATP demands. OXPHOS defects lead to severe human disorders with unexplained tissue specific pathologies. Mitochondrial gene expression is essential for OXPHOS biogenesis since core subunits of the complexes are mitochondrial-encoded. COX14 is required for translation of COX1, the central mitochondrial-encoded subunit of complex IV. Here we describe a COX14 mutant mouse corresponding to a patient with complex IV deficiency. COX14M19I mice display broad tissue-specific pathologies. A hallmark phenotype is severe liver inflammation linked to release of mitochondrial RNA into the cytosol sensed by RIG-1 pathway. We find that mitochondrial RNA release is triggered by increased reactive oxygen species production in the deficiency of complex IV. Additionally, we describe a COA3Y72C mouse, affected in an assembly factor that cooperates with COX14 in early COX1 biogenesis, which displays a similar yet milder inflammatory phenotype. Our study provides insight into a link between defective mitochondrial gene expression and tissue-specific inflammation.