Article
Familial hypertrophic cardiomyopathy-linked mutant troponin T causes stress-induced ventricular tachycardia and Ca2+-dependent action potential remodeling.
Circulation research - 7 Mar 2003
Knollmann Björn C, Kirchhof Paulus, Sirenko Syevda G, Degen Hubertus, Greene Anne E, Schober Tilmann, Mackow Jessica C, Fabritz Larissa, Potter James D, Morad Martin
Abstract excerpt
The cardiac troponin T (TnT) I79N mutation has been linked to familial hypertrophic cardiomyopathy and high incidence of sudden death, despite causing little or no cardiac hypertrophy in patients. Transgenic mice expressing mutant human TnT (I79N-Tg) have increased cardiac contractility, but no ventricular hypertrophy or fibrosis. Enhanced cardiac function has been associated with myofilament Ca2+ sensitization,...
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