Hypertrophic and Dilated Cardiomyopathy-Associated Troponin T Mutations R130C and ΔK210 Oppositely Affect Length-Dependent Calcium Sensitivity of Force Generation.

ABSTRACT: Length-dependent activation of calcium-dependent myocardial force generation provides the basis for the Frank-Starling mechanism. To directly compare the effects of mutations associated with hypertrophic cardiomyopathy and dilated cardiomyopathy, the native troponin complex in skinned trabecular fibers of guinea pigs was exchanged with recombinant heterotrimeric, human, cardiac troponin complexes containing different human cardiac troponin T subunits (hcTnT): hypertrophic cardiomyopathy-associated hcTnT^R130C, dilated cardiomyopathy-associated hcTnT^ΔK210 or the wild type hcTnT (hcTnT^WT) serving as control. Force-calcium relations of exchanged fibers were explored at short fiber length defined as 110% of slack length (L ₀) and long fi

SUBMITTER: Groen M 

PROVIDER: S-EPMC7283609 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature