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- W2763670006 abstract "Significance Mutations in genes encoding sarcomeric proteins are the major cause of primary inherited cardiomyopathies. Troponin T (TnT), encoded by TNNT2 , harbors most of its pathogenic mutants at TNT1 (residues ∼80–180 of TnT). TNT1 is known to interact with tropomyosin (Tm). In this study, we have analyzed TNT1 mutants using in vitro and in silico methods and correlated the results. We also found a striking correlation between binding affinities for Tm and changes in the calcium sensitivity of regulated actomyosin ATPase activities within residues 92–144. These data are consistent with reducing or increasing the affinity of TnT for Tm as the primary cause of cardiomyopathy for mutations in this region, suggesting a smaller Tm binding region." @default.
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- W2763670006 date "2017-10-02" @default.
- W2763670006 modified "2023-09-27" @default.
- W2763670006 title "Molecular mechanisms and structural features of cardiomyopathy-causing troponin T mutants in the tropomyosin overlap region" @default.
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- W2763670006 doi "https://doi.org/10.1073/pnas.1710354114" @default.
- W2763670006 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5651771" @default.
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