ArticleBasic research in cardiology2023
Activation of the integrated stress response rewires cardiac metabolism in Barth syndrome.
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Article in Basic research in cardiology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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18citing papers in PubMed
3.1field-weighted citation impact, top 8% of its field
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Who cites it
18 citing papers in PubMed, 20 citations in OpenAlex.
- Peroxisomal catalase and plasmalogen biosynthesis protect from oxidative stress in Barth syndrome cardiomyopathy.Basic research in cardiology · 2026Article
- Adaptation of OXPHOS biogenesis to cellular requirements.Protein science : a publication of the Protein Society · 2026Review
- Loss of TAFAZZIN leads to perturbation of amino acid metabolism and reduction of collagen synthesis.bioRxiv : the preprint server for biology · 2026Article
- mJournal of cardiothoracic surgery · 2026Article
- Perioperative Metabolomic Profiling in Neonatal Cardiac Surgery Identifies a High-Risk Phenotype for Adverse Outcomes.JACC. Advances · 2026Article
- Identification and verification of biomarkers associated with integrated stress response in heart failure.Scientific reports · 2026Article
- Mitoregulin supports mitochondrial membrane integrity and protects against cardiac ischaemia-reperfusion injury.Cardiovascular research · 2026Article
- Disturbed mitochondrial maturation in cardiolipin remodeling-deficient cardiomyocytes.iScience · 2026Article
- Review
- Adaptive mechanisms in pancreatic islets counteract mitochondrial dysfunction in Barth syndrome.Diabetologia · 2026Article
- Metabolic cardiomyopathies: untangling clinical heterogeneity with human stem-cell derived models.EMBO molecular medicine · 2025Review
- Mechano-energetic uncoupling in heart failure.Nature reviews. Cardiology · 2025Review
- Article
- A murine model of Barth syndrome recapitulates human cardiac and skeletal muscle phenotypes.Disease models & mechanisms · 2025Article
- Maximizing ISRIB Potential Requires Addressing Specificity, Long-term Safety, and Disease-specific Considerations.Current medicinal chemistry · 2025Review
- The attenuated hepatic clearance of propionate increases cardiac oxidative stress in propionic acidemia.Basic research in cardiology · 2024Article
- Review
- Tafazzin deficiency causes substantial remodeling in the lipidome of a mouse model of Barth Syndrome cardiomyopathy.Frontiers in molecular medicine · 2024Article
Corrections and comments
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Authors and funding
25 authors at 10 institutions in 4 countries.
Ilona KutschkaDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Edoardo BerteroDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Christina WasmusDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Ke XiaoInstitute of Molecular and Translational Therapeutic Strategies, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
Lifeng YangShanghai Institute of Nutrition and Health, Chinese Academy of Sciences, 320 Yueyang Rd, Shanghai, 200031, China.
Xinyu ChenDepartment of Nuclear Medicine, University Clinic Würzburg, Oberdürrbacher Strasse 6, 97080, Würzburg, Germany.
Yasuhiro OshimaDepartment of Nuclear Medicine, University Clinic Würzburg, Oberdürrbacher Strasse 6, 97080, Würzburg, Germany.
Marcus FischerDivision of Pediatric Cardiology and Intensive Care, University Hospital LMU Munich, Marchioninistr. 15, 81377, Munich, Germany.
Manuela ErkDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Berkan ArslanDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Lin AlhasanDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Daria GrosserDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Katharina J ErmerDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Alexander NickelDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Michael KohlhaasDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Hanna EberlDepartment for Pharmacology and Toxicology, University of Würzburg, Versbacher Strasse 9, 97078, Würzburg, Germany.
Sabine RebsDepartment for Pharmacology and Toxicology, University of Würzburg, Versbacher Strasse 9, 97078, Würzburg, Germany.
Katrin Streckfuss-BömekeDepartment for Pharmacology and Toxicology, University of Würzburg, Versbacher Strasse 9, 97078, Würzburg, Germany.
Werner SchmitzDepartment of Biochemistry and Molecular Biology, University of Würzburg, Am Hubland, 97074, Würzburg, Germany.
Peter RehlingUniversity Göttingen, Institute of Biochemistry and Molecular Cell Biology, Humboldtallee 23, 37072, Göttingen, Germany.
Thomas ThumInstitute of Molecular and Translational Therapeutic Strategies, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
Takahiro HiguchiDepartment of Nuclear Medicine, University Clinic Würzburg, Oberdürrbacher Strasse 6, 97080, Würzburg, Germany.
Joshua RabinowitzLewis-Sigler Institute for Integrative Genomics, Princeton University, Princeton, NJ, 08544, USA.
Christoph MaackDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany.
Jan DudekDepartment of Translational Research, Comprehensive Heart Failure Center (CHFC), University Clinic Würzburg, Am Schwarzenberg 15, Haus A15, 97078, Würzburg, Germany. Dudek_J@ukw.de.
Universitätsklinikum Würzburg · DEUniversity of Würzburg · DEFraunhofer Institute for Toxicology and Experimental Medicine · DEGerman Centre for Cardiovascular Research · DELudwig-Maximilians-Universität München · DEMedizinische Hochschule Hannover · DEOspedale Policlinico San Martino · ITPrinceton University · USShanghai Institute of Nutrition and Health · CNUniversity of Göttingen · DE
Funding
European Research Council 101054460
Abstract
Barth Syndrome (BTHS) is an inherited cardiomyopathy caused by defects in the mitochondrial transacylase TAFAZZIN (Taz), required for the synthesis of the phospholipid cardiolipin. BTHS is characterized by heart failure, increased propensity for arrhythmias and a blunted inotropic reserve. Defects in Ca
Indexed as
Barth SyndromeAnimalsAntioxidantsCystineFatty AcidsGlutamatesGlutathioneMiceAntioxidantsCystineFatty AcidsGlutamatesGlutathioneAmino acidBarth syndromeFatty acid oxidationMetabolismMitochondriaOxidative stress
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.