Structural destabilization of tropomyosin induced by the cardiomyopathy‐linked mutation R21H

The missense mutation R21H in striated muscle tropomyosin is associated with hypertrophic cardiomyopathy, a genetic cardiac disease and a leading cause of sudden cardiac death in young people. Tropomyosin adopts conformation of a coiled coil which is critical for regulation of muscle contraction. In...

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Veröffentlicht in:	Protein science 2018-02, Vol.27 (2), p.498-508
Hauptverfasser:	Ly, Thu, Krieger, Inna, Tolkatchev, Dmitri, Krone, Cheyenna, Moural, Timothy, Samatey, Fadel A., Kang, ChulHee, Kostyukova, Alla S.
Format:	Artikel
Sprache:	eng
Schlagworte:	Affinity Binding Binding Sites Calorimetry Cardiomyopathy Cardiomyopathy, Hypertrophic - genetics Circular Dichroism Coils Conformation Coronary artery disease Crystal structure Crystallography, X-Ray Destabilization Dichroism Filaments Heart diseases Humans hypertrophic cardiomyopathy isothermal titration calorimetry leiomodin Microfilament Proteins - metabolism Missense mutation Models, Molecular Molecular dynamics Molecular Dynamics Simulation Muscle contraction Muscle Proteins - metabolism Muscles Mutation Mutation, Missense Protein Binding Protein Stability Protein Structure, Secondary Simulation Skeletal muscle Structural integrity Titration Titration calorimetry tropomodulin Tropomodulin - metabolism Tropomyosin Tropomyosin - chemistry Tropomyosin - genetics Tropomyosin - metabolism Young adults
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