Structural Modelling of KCNQ1 and KCNH2 Double Mutant Proteins, Identified in Two Severe Long QT Syndrome Cases, Reveals New Insights into Cardiac Channelopathies

Structural Modelling of KCNQ1 and KCNH2 Double Mutant Proteins, Identified in Two Severe Long QT Syndrome Cases, Reveals New Insights into Cardiac Channelopathies

Congenital long QT syndrome (LQTS) is a cardiac channelopathy characterized by a prolongation of the QT interval and T-wave abnormalities, caused, in most cases, by mutations in KCNQ1, KCNH2, and SCN5A. Although the predominant pattern of LQTS inheritance is autosomal dominant, compound heterozygous...

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Bibliographic Details
Main Authors: William A. Agudelo, Sebastian Ramiro Gil-Quiñones, Alejandra Fonseca, Alvaro Arenas, Laura Castro, Diana Carolina Sierra-Díaz, Manuel A. Patarroyo, Paul Laissue, Carlos F. Suárez, Rodrigo Cabrera
Format: Article
Language:English
Published: MDPI AG 2021-11-01
Series:International Journal of Molecular Sciences
Subjects:
long QT syndrome
<i>KCNQ1</i>
<i>KCNH2</i>
cardiac channelopathies-computational modelling
Online Access:https://www.mdpi.com/1422-0067/22/23/12861

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https://www.mdpi.com/1422-0067/22/23/12861

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