Polymorphic ventricular tachycardia and abnormal Ca handling in very-long-chain acyl-CoA dehydrogenase null mice
نویسندگان
چکیده
Andreas A. Werdich, Franz Baudenbacher, Igor Dzhura, Loice H. Jeyakumar, Prince J. Kannankeril, Sidney Fleischer, Alison LeGrone, Dejan Milatovic, Michael Aschner, Arnold W. Strauss, Mark E. Anderson, and Vernat J. Exil Division of Cardiology, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville; Division of Gastroenterology, Vanderbilt University Medical Center, Nashville; Departments of Biomedical Engineering and Biological Sciences, Vanderbilt University, Nashville, Tennessee; and Division of Cardiology, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, Iowa
منابع مشابه
Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice.
Patients with mutations in the mitochondrial very-long-chain acyl-CoA dehydrogenase (VLCAD) gene are at risk for cardiomyopathy, myocardial dysfunction, ventricular tachycardia (VT), and sudden cardiac death. The mechanism is not known. Here we report a novel mechanism of VT in mice lacking VLCAD (VLCAD(-/-)). These mice exhibited polymorphic VT and increased incidence of VT after isoproterenol...
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Patients with very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency frequently present cardiomyopathy and heartbeat disorders. However, the underlying factors, which may be of cardiac or extra cardiac origins, remain to be elucidated. In this study, we tested for metabolic and functional alterations in the heart from 3- and 7-mo-old VLCAD null mice and their littermate counterparts, using v...
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Very long-chain acyl-CoA dehydrogenase (VLCAD)-deficiency is the most common long-chain fatty acid oxidation disorder presenting with heterogeneous phenotypes. Similar to many patients with VLCADD, VLCAD-deficient mice (VLCAD(-/-)) remain asymptomatic over a long period of time. In order to identify the involved compensatory mechanisms, wild-type and VLCAD(-/-) mice were fed one year either wit...
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