Visualizing Mutation-Specific Differences in the Trafficking-Deficient Phenotype of Kv11.1 Proteins Linked to Long QT Syndrome Type 2

Visualizing Mutation-Specific Differences in the Trafficking-Deficient Phenotype of Kv11.1 Proteins Linked to Long QT Syndrome Type 2

KCNH2 encodes the Kv11.1 α-subunit that underlies the rapidly activating delayed-rectifier K+ current in the heart. Loss-of-function KCNH2 mutations cause long QT syndrome type 2 (LQT2), and most LQT2-linked missense mutations inhibit the trafficking of Kv11.1 channel protein to the cell surface mem...

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Bibliographic Details
Main Authors: Allison R. Hall, Corey L. Anderson, Jennifer L. Smith, Tooraj Mirshahi, Claude S. Elayi, Craig T. January, Brian P. Delisle
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-05-01
Series:Frontiers in Physiology
Subjects:
K+ channel
trafficking
long QT syndrome
arrhythmia
Kv11.1
hERG
Online Access:https://www.frontiersin.org/article/10.3389/fphys.2018.00584/full

Internet

https://www.frontiersin.org/article/10.3389/fphys.2018.00584/full

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