000271329 001__ 271329
000271329 005__ 20250127091623.0
000271329 0247_ $$2pmc$$apmc:PMC11319346
000271329 0247_ $$2doi$$a10.1038/s41467-024-51109-y
000271329 0247_ $$2pmid$$apmid:39134548
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000271329 037__ $$aDZNE-2024-01031
000271329 041__ $$aEnglish
000271329 082__ $$a500
000271329 1001_ $$00000-0001-8331-9874$$aAich, Abhishek$$b0
000271329 245__ $$aDefective mitochondrial COX1 translation due to loss of COX14 function triggers ROS-induced inflammation in mouse liver.
000271329 260__ $$a[London]$$bNature Publishing Group UK$$c2024
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000271329 520__ $$aMitochondrial oxidative phosphorylation (OXPHOS) fuels cellular ATP demands. OXPHOS defects lead to severe human disorders with unexplained tissue specific pathologies. Mitochondrial gene expression is essential for OXPHOS biogenesis since core subunits of the complexes are mitochondrial-encoded. COX14 is required for translation of COX1, the central mitochondrial-encoded subunit of complex IV. Here we describe a COX14 mutant mouse corresponding to a patient with complex IV deficiency. COX14M19I mice display broad tissue-specific pathologies. A hallmark phenotype is severe liver inflammation linked to release of mitochondrial RNA into the cytosol sensed by RIG-1 pathway. We find that mitochondrial RNA release is triggered by increased reactive oxygen species production in the deficiency of complex IV. Additionally, we describe a COA3Y72C mouse, affected in an assembly factor that cooperates with COX14 in early COX1 biogenesis, which displays a similar yet milder inflammatory phenotype. Our study provides insight into a link between defective mitochondrial gene expression and tissue-specific inflammation.
000271329 536__ $$0G:(DE-HGF)POF4-352$$a352 - Disease Mechanisms (POF4-352)$$cPOF4-352$$fPOF IV$$x0
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000271329 650_7 $$2NLM Chemicals$$aReactive Oxygen Species
000271329 650_7 $$0EC 1.9.3.1$$2NLM Chemicals$$aElectron Transport Complex IV
000271329 650_7 $$0EC 3.6.1.-$$2NLM Chemicals$$aDdx58 protein, mouse
000271329 650_7 $$2NLM Chemicals$$aRNA, Mitochondrial
000271329 650_7 $$0EC 1.14.99.1$$2NLM Chemicals$$aPtgs1 protein, mouse
000271329 650_7 $$2NLM Chemicals$$aMitochondrial Proteins
000271329 650_7 $$0EC 3.6.4.13$$2NLM Chemicals$$aDEAD-box RNA Helicases
000271329 650_7 $$0EC 3.6.4.13$$2NLM Chemicals$$aDEAD Box Protein 58
000271329 650_7 $$2NLM Chemicals$$aMembrane Proteins
000271329 650_7 $$0EC 1.14.99.1$$2NLM Chemicals$$aCyclooxygenase 1
000271329 650_2 $$2MeSH$$aAnimals
000271329 650_2 $$2MeSH$$aReactive Oxygen Species: metabolism
000271329 650_2 $$2MeSH$$aMice
000271329 650_2 $$2MeSH$$aLiver: metabolism
000271329 650_2 $$2MeSH$$aLiver: pathology
000271329 650_2 $$2MeSH$$aInflammation: metabolism
000271329 650_2 $$2MeSH$$aInflammation: genetics
000271329 650_2 $$2MeSH$$aInflammation: pathology
000271329 650_2 $$2MeSH$$aElectron Transport Complex IV: metabolism
000271329 650_2 $$2MeSH$$aElectron Transport Complex IV: genetics
000271329 650_2 $$2MeSH$$aOxidative Phosphorylation
000271329 650_2 $$2MeSH$$aMitochondria: metabolism
000271329 650_2 $$2MeSH$$aHumans
000271329 650_2 $$2MeSH$$aRNA, Mitochondrial: genetics
000271329 650_2 $$2MeSH$$aRNA, Mitochondrial: metabolism
000271329 650_2 $$2MeSH$$aMale
000271329 650_2 $$2MeSH$$aMitochondrial Proteins: metabolism
000271329 650_2 $$2MeSH$$aMitochondrial Proteins: genetics
000271329 650_2 $$2MeSH$$aProtein Biosynthesis
000271329 650_2 $$2MeSH$$aMice, Inbred C57BL
000271329 650_2 $$2MeSH$$aDEAD-box RNA Helicases: metabolism
000271329 650_2 $$2MeSH$$aDEAD-box RNA Helicases: genetics
000271329 650_2 $$2MeSH$$aFemale
000271329 650_2 $$2MeSH$$aMutation
000271329 650_2 $$2MeSH$$aDEAD Box Protein 58
000271329 650_2 $$2MeSH$$aMembrane Proteins
000271329 650_2 $$2MeSH$$aCyclooxygenase 1
000271329 7001_ $$00009-0008-3572-0223$$aBoshnakovska, Angela$$b1
000271329 7001_ $$aWitte, Steffen$$b2
000271329 7001_ $$aGall, Tanja$$b3
000271329 7001_ $$aUnthan-Fechner, Kerstin$$b4
000271329 7001_ $$aYousefi, Roya$$b5
000271329 7001_ $$00000-0001-5025-0268$$aChowdhury, Arpita$$b6
000271329 7001_ $$aDahal, Drishan$$b7
000271329 7001_ $$0P:(DE-2719)9001018$$aMethi, Aditi$$b8$$udzne
000271329 7001_ $$aKaufmann, Svenja$$b9
000271329 7001_ $$00000-0001-8284-954X$$aSilbern, Ivan$$b10
000271329 7001_ $$00000-0003-4675-8995$$aProchazka, Jan$$b11
000271329 7001_ $$00000-0001-7358-3268$$aNichtova, Zuzana$$b12
000271329 7001_ $$aPalkova, Marcela$$b13
000271329 7001_ $$aRaishbrook, Miles$$b14
000271329 7001_ $$00000-0001-6497-6094$$aKoubkova, Gizela$$b15
000271329 7001_ $$00000-0002-3352-392X$$aSedlacek, Radislav$$b16
000271329 7001_ $$00000-0003-1156-2976$$aTröder, Simon E$$b17
000271329 7001_ $$aZevnik, Branko$$b18
000271329 7001_ $$00000-0003-2970-6894$$aRiedel, Dietmar$$b19
000271329 7001_ $$aMichanski, Susann$$b20
000271329 7001_ $$00000-0002-2902-7165$$aMöbius, Wiebke$$b21
000271329 7001_ $$aStröbel, Philipp$$b22
000271329 7001_ $$aLüchtenborg, Christian$$b23
000271329 7001_ $$00000-0002-4636-1827$$aGiavalisco, Patrick$$b24
000271329 7001_ $$00000-0003-1837-5233$$aUrlaub, Henning$$b25
000271329 7001_ $$0P:(DE-2719)2000047$$aFischer, Andre$$b26$$udzne
000271329 7001_ $$00000-0002-3477-8270$$aBrügger, Britta$$b27
000271329 7001_ $$00000-0002-8028-3121$$aJakobs, Stefan$$b28
000271329 7001_ $$00000-0001-5661-5272$$aRehling, Peter$$b29
000271329 773__ $$0PERI:(DE-600)2553671-0$$a10.1038/s41467-024-51109-y$$gVol. 15, no. 1, p. 6914$$n1$$p6914$$tNature Communications$$v15$$x2041-1723$$y2024
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