000280737 001__ 280737
000280737 005__ 20250905102146.0
000280737 0247_ $$2doi$$a10.5281/ZENODO.10640936
000280737 0247_ $$2doi$$a10.5281/zenodo.10640936
000280737 0247_ $$2doi$$a10.5281/zenodo.10640935
000280737 037__ $$aDZNE-2025-00958
000280737 041__ $$aEnglish
000280737 1001_ $$aAich, Abhishek$$b0
000280737 245__ $$aDataset: Defective mitochondrial COX1 translation due to loss of COX14 function triggers ROS-induced inflammation in liver
000280737 260__ $$bZenodo$$c2024
000280737 3367_ $$2BibTeX$$aMISC
000280737 3367_ $$0PUB:(DE-HGF)32$$2PUB:(DE-HGF)$$aDataset$$bdataset$$mdataset$$s1756976126_4383
000280737 3367_ $$026$$2EndNote$$aChart or Table
000280737 3367_ $$2DataCite$$aDataset
000280737 3367_ $$2ORCID$$aDATA_SET
000280737 3367_ $$2DINI$$aResearchData
000280737 520__ $$aData Content In this data repository Ultra High-Performance Chromatography (UPLC) and Ion Chromatography (IC)- high resolution mass spectrometry (HRMS) metabolomic data is provided. The data consists of all analyzed raw files and the associated peak picking files (TraceFinder 5.1, Thermo Fisher Scientific). The data with the abreviation 'Bz_QE_Plus' contains the UPLC-MS data of bezoylchloride derivatized amines, phenols, thiols, and some alcohols measured on a Q-Exactive Plus mass spectrometer), while the files with the abbreviation 'IC_HF_TF' contain the anion chromatographic data measured on a Q-Exactive Plus coupled to an Integrion ion chroatograph. The provided data is associated to an article submitted to Nature Communications (“Defective mitochondrial COX1 translation due to loss of COX14 function triggers ROS-induced inflammation in liver”), describing the analysis of COX14 and COA3 mutants (see abstract below). The sample and data analysis section, which is also provided in the supplementary section of the above mentioned article, are also attached as, doc files to the deposited data.Abstract of the article 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 generated 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 generated a COA3Y72C mouse, affected in an assembly factor in early COX1 biogenesis, which displayed a similar yet milder inflammatory phenotype. Our study provides mechanistic insight into how defective mitochondrial gene expression causes tissue-specific inflammation.
000280737 536__ $$0G:(DE-HGF)POF4-352$$a352 - Disease Mechanisms (POF4-352)$$cPOF4-352$$fPOF IV$$x0
000280737 588__ $$aDataset connected to DataCite
000280737 7001_ $$aPatrick, Giavalisco$$b1
000280737 7001_ $$aRehling, Peter$$b2
000280737 7001_ $$aAich, Abhishek$$b3$$eData Collector
000280737 7001_ $$aBoshnakovska, Angela$$b4$$eData Collector
000280737 7001_ $$aWitte, Steffen$$b5$$eData Collector
000280737 7001_ $$aGall, Tanja$$b6$$eData Collector
000280737 7001_ $$aUnthan-Fechner, Kerstin$$b7$$eData Collector
000280737 7001_ $$aYousefi, Roya$$b8$$eData Collector
000280737 7001_ $$aChowdhury, Arpita$$b9$$eData Collector
000280737 7001_ $$aDahal, Drishan$$b10$$eData Collector
000280737 7001_ $$0P:(DE-2719)9001018$$aMethi, Aditi$$b11$$eData Collector$$udzne
000280737 7001_ $$aKaufmann, Svenja$$b12$$eData Collector
000280737 7001_ $$aSilbern, Ivan$$b13$$eData Collector
000280737 7001_ $$aProchazka, Jan$$b14$$eData Collector
000280737 7001_ $$aNichtova, Zusana$$b15$$eData Collector
000280737 7001_ $$aPalkova, Marcela$$b16$$eData Collector
000280737 7001_ $$aRaishbrook, Miles$$b17$$eData Collector
000280737 7001_ $$aKoublova, Gizela$$b18$$eData Collector
000280737 7001_ $$aSedlacek, Radislav$$b19$$eData Collector
000280737 7001_ $$aTröder, Simon E.$$b20$$eData Collector
000280737 7001_ $$aZevnik, Branko$$b21$$eData Collector
000280737 7001_ $$aRiedel, Dietmar$$b22$$eData Collector
000280737 7001_ $$aMichanski, Susann$$b23$$eData Collector
000280737 7001_ $$aMöbius, Wiebke$$b24$$eData Collector
000280737 7001_ $$aStröbel, Philipp$$b25$$eData Collector
000280737 7001_ $$aLüchtenborg, Christian$$b26$$eData Collector
000280737 7001_ $$aGiavalisco, Patrick$$b27$$eData Collector
000280737 7001_ $$aUrlaub, Henning$$b28$$eData Collector
000280737 7001_ $$0P:(DE-2719)2000047$$aFischer, Andre$$b29$$eData Collector$$udzne
000280737 7001_ $$aBrügger, Britta$$b30$$eData Collector
000280737 7001_ $$aJakobs, Stefan$$b31$$eData Collector
000280737 7001_ $$aRehling, Peter$$b32$$eContact Person
000280737 773__ $$a10.5281/zenodo.10640935
000280737 7870_ $$0DZNE-2024-01031$$aAich, Abhishek et.al.$$d[London] : Nature Publishing Group UK, 2024$$iRelatedTo$$tDefective mitochondrial COX1 translation due to loss of COX14 function triggers ROS-induced inflammation in mouse liver.
000280737 909CO $$ooai:pub.dzne.de:280737$$pVDB
000280737 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)9001018$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b11$$kDZNE
000280737 9101_ $$0I:(DE-HGF)0$$6P:(DE-2719)2000047$$aExternal Institute$$b29$$kExtern
000280737 9131_ $$0G:(DE-HGF)POF4-352$$1G:(DE-HGF)POF4-350$$2G:(DE-HGF)POF4-300$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lNeurodegenerative Diseases$$vDisease Mechanisms$$x0
000280737 9201_ $$0I:(DE-2719)1410002$$kAG Fischer$$lEpigenetics and Systems Medicine in Neurodegenerative Diseases$$x0
000280737 980__ $$adataset
000280737 980__ $$aVDB
000280737 980__ $$aI:(DE-2719)1410002
000280737 980__ $$aUNRESTRICTED