Key result
In a transgenic rat model of FHC, a C-terminal truncation of the cardiac TnT molecule impaired force-generating capacity and decreased Ca(2+) sensitivity while increasing tension-dependent ATP utilization.
A truncation mutation in cardiac troponin T impairs force generation and increases ATP utilization, suggesting energy compromise contributes to the pathogenesis of familial hypertrophic cardiomyopathy.
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Implicates energy inefficiency in FHC pathogenesis; leaves open human translation and therapeutic relevance.
Frey et al. (2006) studied Familial hypertrophic cardiomyopathy (FHC). Expression of a human TnT molecule with a truncation mutation (DEL-TnT) vs. Rats expressing wild-type human TnT and nontransgenic rats was evaluated on Force of contraction, intracellular Ca(2+) transients, myofibrillar Ca(2+) sensitivity, and myofibrillar ATP utilization. In a transgenic rat model of FHC, a C-terminal truncation of the cardiac TnT molecule impaired force-generating capacity and decreased Ca(2+) sensitivity while increasing tension-dependent ATP utilization.
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