Characterization of the novel mutant A78T-HERG from a long QT syndrome type 2 patient: Instability of the mutant protein and stabilization by heat shock factor 1

Characterization of the novel mutant A78T-HERG from a long QT syndrome type 2 patient: Instability of the mutant protein and stabilization by heat shock factor 1

Background: The human ether-a-go-go-related gene (HERG) encodes the α-subunit of rapidly activating delayed-rectifier potassium channels. Mutations in this gene cause long QT syndrome type 2 (LQT2). In most cases, mutations reduce the stability of the channel protein, which can be restored by heat s...

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Bibliographic Details
Main Authors: Takehito Kondo, Ichiro Hisatome, Shouichi Yoshimura, Endang Mahati, Tomomi Notsu, Peili Li, Kazuhiko Iitsuka, Masaru Kato, Kazuyoshi Ogura, Junichiro Miake, Takeshi Aiba, Wataru Shimizu, Yasutaka Kurata, Shinji Sakata, Naoe Nakasone, Haruaki Ninomiya, Akira Nakai, Katsumi Higaki, Yasushi Kawata, Yasuaki Shirayoshi, Akio Yoshida, Kazuhiro Yamamoto
Format: Article
Language:English
Published: Wiley 2016-10-01
Series:Journal of Arrhythmia
Subjects:
Long QT syndrome
Human ether-a-go-go-related gene
Heat shock factor
Online Access:http://www.sciencedirect.com/science/article/pii/S188042761500143X

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http://www.sciencedirect.com/science/article/pii/S188042761500143X

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