Molecular Mechanisms of Muscle Weakness Associated with E173A Mutation in Tpm3.12. Troponin Ca<sup>2+</sup> Sensitivity Inhibitor W7 Can Reduce the Damaging Effect of This Mutation

Molecular Mechanisms of Muscle Weakness Associated with E173A Mutation in Tpm3.12. Troponin Ca<sup>2+</sup> Sensitivity Inhibitor W7 Can Reduce the Damaging Effect of This Mutation

Substitution of Ala for Glu residue in position 173 of γ-tropomyosin (Tpm3.12) is associated with muscle weakness. Here we observe that this mutation increases myofilament Ca<sup>2+</sup>-sensitivity and inhibits in vitro actin-activated ATPase activity of myosin subfragment-1 at high Ca...

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Bibliographic Details
Main Authors: Yurii S. Borovikov, Armen O. Simonyan, Stanislava V. Avrova, Vladimir V. Sirenko, Charles S. Redwood, Olga E. Karpicheva
Format: Article
Language:English
Published: MDPI AG 2020-06-01
Series:International Journal of Molecular Sciences
Subjects:
tropomyosin
mutations in tropomyosin
muscle weakness
congenital myopathy
Ca<sup>2+</sup>-sensitivity of myofilament
ATPase activity of myosin
Online Access:https://www.mdpi.com/1422-0067/21/12/4421

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https://www.mdpi.com/1422-0067/21/12/4421

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