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@MISC{Aich:280737,
author = {Aich, Abhishek and Patrick, Giavalisco and Rehling, Peter},
othercontributors = {Aich, Abhishek and Boshnakovska, Angela and Witte, Steffen
and Gall, Tanja and Unthan-Fechner, Kerstin and Yousefi,
Roya and Chowdhury, Arpita and Dahal, Drishan and Methi,
Aditi and Kaufmann, Svenja and Silbern, Ivan and Prochazka,
Jan and Nichtova, Zusana and Palkova, Marcela and
Raishbrook, Miles and Koublova, Gizela and Sedlacek,
Radislav and Tröder, Simon E. and Zevnik, Branko and
Riedel, Dietmar and Michanski, Susann and Möbius, Wiebke
and Ströbel, Philipp and Lüchtenborg, Christian and
Giavalisco, Patrick and Urlaub, Henning and Fischer, Andre
and Brügger, Britta and Jakobs, Stefan and Rehling, Peter},
title = {{D}ataset: {D}efective mitochondrial {COX}1 translation due
to loss of {COX}14 function triggers {ROS}-induced
inflammation in liver},
publisher = {Zenodo},
reportid = {DZNE-2025-00958},
year = {2024},
abstract = {Data 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.},
cin = {AG Fischer},
cid = {I:(DE-2719)1410002},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)32},
doi = {10.5281/zenodo.10640935},
url = {https://pub.dzne.de/record/280737},
}