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Autor
Aich, Abhishek 1 Boshnakovska, Angela 1 Brügger, Britta 1 Chowdhury, Arpita 1 Cyganek, Lukas 1 Dudek, Jan 1 Fischer, André 1 Katschinski, Dörthe M 1 Koubkova, Gizela 1 Lindovsky, Jiri 1 Lüchtenborg, Christian 1 Methi, Aditi 1 Nichtova, Zuzana 1 Prochazka, Jan 1 Rehling, Peter 1 Sedlacek, Radislav 1 Silbern, Ivan 1 Urlaub, Henning 1 Vergel Leon, Ana Maria 1 Wachs, Dominic 1
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Pracoviště
Cluster of Excellence Multiscale Bioi... 1 Czech Centre for Phenogenomics Instit... 1 DZHK partner site Göttingen Göttingen... 1 Department for Epigenetics and System... 1 Department of Cardiovascular Physiolo... 1 Department of Cellular Biochemistry U... 1 Department of Psychiatry and Psychoth... 1 Heidelberg University Biochemistry Ce... 1 Institute for Clinical Chemistry Univ... 1 Max Planck Institute for Multidiscipl... 1 Stem Cell Unit Clinic for Cardiology ... 1 The Bioanalytical Mass Spectrometry G... 1
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Autor
Aich, Abhishek 1 Boshnakovska, Angela 1 Brügger, Britta 1 Chowdhury, Arpita 1 Cyganek, Lukas 1 Dudek, Jan 1 Fischer, André 1 Katschinski, Dörthe M 1 Koubkova, Gizela 1 Lindovsky, Jiri 1 Lüchtenborg, Christian 1 Methi, Aditi 1 Nichtova, Zuzana 1 Prochazka, Jan 1 Rehling, Peter 1 Sedlacek, Radislav 1 Silbern, Ivan 1 Urlaub, Henning 1 Vergel Leon, Ana Maria 1 Wachs, Dominic 1
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Pracoviště
Cluster of Excellence Multiscale Bioi... 1 Czech Centre for Phenogenomics Instit... 1 DZHK partner site Göttingen Göttingen... 1 Department for Epigenetics and System... 1 Department of Cardiovascular Physiolo... 1 Department of Cellular Biochemistry U... 1 Department of Psychiatry and Psychoth... 1 Heidelberg University Biochemistry Ce... 1 Institute for Clinical Chemistry Univ... 1 Max Planck Institute for Multidiscipl... 1 Stem Cell Unit Clinic for Cardiology ... 1 The Bioanalytical Mass Spectrometry G... 1
- Formát
- Publikační typ
- Check Tag
- Kategorie
- Jazyk
- Země
- Časopis/zdroj
- Dostupnost
- Vlastník
- Chowdhury, Arpita
- Boshnakovska, Angela
- Aich, Abhishek
- Methi, Aditi
- Vergel Leon, Ana Maria
- Silbern, Ivan
- Lüchtenborg, Christian
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Cyganek, Lukas
Autor Cyganek, Lukas Cluster of Excellence "Multiscale Bioimaging: from Molecular Machines to Networks of Excitable Cells" (MBExC), University of Göttingen, Göttingen, Germany DZHK (German Center for Cardiovascular Research) partner site Göttingen, Göttingen, Germany Stem Cell Unit, Clinic for Cardiology and Pneumology, University Medical Center Göttingen, Georg-August University Göttingen, Göttingen, Germany
- Prochazka, Jan
- Sedlacek, Radislav
NLK
Directory of Open Access Journals
od 2009
Free Medical Journals
od 2009
PubMed Central
od 2009
Europe PubMed Central
od 2009
ProQuest Central
od 2009-04-01
Open Access Digital Library
od 2009-01-01
Open Access Digital Library
od 2009-01-01
Open Access Digital Library
od 2009-01-01
Medline Complete (EBSCOhost)
od 2011-01-01
Health & Medicine (ProQuest)
od 2009-04-01
ROAD: Directory of Open Access Scholarly Resources
od 2008
PubMed
37533404
DOI
10.15252/emmm.202317399
Knihovny.cz E-zdroje
Mitochondria are central for cellular metabolism and energy supply. Barth syndrome (BTHS) is a severe disorder, due to dysfunction of the mitochondrial cardiolipin acyl transferase tafazzin. Altered cardiolipin remodeling affects mitochondrial inner membrane organization and function of membrane proteins such as transporters and the oxidative phosphorylation (OXPHOS) system. Here, we describe a mouse model that carries a G197V exchange in tafazzin, corresponding to BTHS patients. TAZG197V mice recapitulate disease-specific pathology including cardiac dysfunction and reduced oxidative phosphorylation. We show that mutant mitochondria display defective fatty acid-driven oxidative phosphorylation due to reduced levels of carnitine palmitoyl transferases. A metabolic switch in ATP production from OXPHOS to glycolysis is apparent in mouse heart and patient iPSC cell-derived cardiomyocytes. An increase in glycolytic ATP production inactivates AMPK causing altered metabolic signaling in TAZG197V . Treatment of mutant cells with AMPK activator reestablishes fatty acid-driven OXPHOS and protects mice against cardiac dysfunction.
- MeSH
- adenosintrifosfát MeSH
- Barthův syndrom * metabolismus patologie MeSH
- glykolýza MeSH
- kardiolipiny metabolismus MeSH
- mastné kyseliny metabolismus MeSH
- myši MeSH
- proteinkinasy aktivované AMP metabolismus MeSH
- zvířata MeSH
- Check Tag
- myši MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
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