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المؤلفون: Arianna F. Anzmann, Olivia L. Sniezek, Steven M. Claypool, Alexandra Pado, Lauren R. DeVine, Hilary J. Vernon, Frédéric M. Vaz, Robert N. Cole, Anne Le, Simion Kreimer, Veronica F. Busa, Brian J. Kirsch
المساهمون: Laboratory Genetic Metabolic Diseases, Amsterdam Gastroenterology Endocrinology Metabolism, APH - Personalized Medicine, APH - Methodology
المصدر: Journal of biological chemistry, 297(3):101005. American Society for Biochemistry and Molecular Biology Inc.
The Journal of Biological Chemistryمصطلحات موضوعية: Proteomics, mitochondrial metabolism, OXPHOS, oxidative phosphorylation, Tafazzin, Biochemistry, chemistry.chemical_compound, PARL, presenilin-associated rhomboid-like protein, Cardiolipin, Inner mitochondrial membrane, biology, BN-PAGE, blue native-PAGE, PARL, Barth syndrome, CII, complex II, MQC, mitochondrial quality control, Cell biology, Mitochondria, Mitochondrial respiratory chain, BEL, bromoenol lactone, PGAM5, phosphoglycerate mutase 5, IMM, inner mitochondrial membrane, Research Article, CL, cardiolipin, Cardiolipins, sgRNA, single-guide RNA, HEK293, human embryonic kidney 293 cell, Oxidative phosphorylation, KEGG, Kyoto Encyclopedia of Genes and Genomes, Small Molecule Libraries, cDNA, complementary DNA, TAZ, TAFFAZIN, BTHS, Barth syndrome, CCCP, carbonyl cyanide m-chlorophenyl hydrazine, GO, Gene Ontology, medicine, Humans, CI, complex I, Molecular Biology, MLCL, monolysocardiolipin, PBST, PBS with 0.2% (v/v) Tween-20, Monolysocardiolipin, CIV, complex IV, Cell Biology, FC, fold change, medicine.disease, HEK293 Cells, chemistry, Barth Syndrome, Lipidomics, biology.protein, cardiolipin, Acyltransferases
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8513153ad63be4ec2a55413a2dd75dea
https://pure.amc.nl/en/publications/diverse-mitochondrial-abnormalities-in-a-new-cellular-model-of-taffazzin-deficiency-are-remediated-by-cardiolipininteracting-small-molecules(0d7f8d5c-d63e-47b4-9cdd-35b501bb7f6b).html -
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المؤلفون: Paulína Káňovičová, Petra Čermáková, Dominika Kubalová, Lenka Bábelová, Petra Veselá, Martin Valachovič, Jakub Zahumenský, Anton Horváth, Jan Malínský, Mária Balážová
المصدر: The Journal of Biological Chemistry
مصطلحات موضوعية: WT, wild type, tafazzin, CL, cardiolipin, TAG, triacylglycerols, VPA, valproic acid, Cardiolipins, Pgc1, phosphatidylglycerol phospholipase C, Pgs1, phosphatidylglycerolphosphate synthase, Saccharomyces cerevisiae, PE, phosphatidylethanolamine, PG, phosphatidylglycerol, Biochemistry, Erg1, squalene epoxidase, PI, phosphatidylinositol, ETS capacity, maximum electron transfer system capacity, PC, phosphatidylcholine, valproic acid, BTHS, Barth syndrome, Crd1, cardiolipin synthase, Humans, phosphatidylglycerol, PA, phosphatidic acid, RCI, respiratory control index, Molecular Biology, MLCL, monolysocardiolipin, OXPHOS capacity, oxidative phosphorylation capacity, SE, sterol esters, Taz1, lyso-phosphatidylcholine acyltransferase, Phosphatidylglycerols, Cell Biology, PS, phosphatidylserine, mitochondria, Phenotype, Erg11, lanosterol 14-alpha-demethylase, PGP, phosphatidylglycerol phosphate, Barth Syndrome, lipids (amino acids, peptides, and proteins), DAG, diacylglycerol, Acyltransferases, Transcription Factors, Research Article