Article
MOG1 rescues defective trafficking of Na(v)1.5 mutations in Brugada syndrome and sick sinus syndrome.
Circulation. Arrhythmia and electrophysiology - 1 Apr 2013
Chakrabarti Susmita, Wu Xiaofen, Yang Zhaogang, Wu Ling, Yong Sandro L, Zhang Cuntai, Hu Keli, Wang Qing K, Chen Qiuyun
Abstract excerpt
BACKGROUND: Loss-of-function mutations in Na(v)1.5 cause sodium channelopathies, including Brugada syndrome, dilated cardiomyopathy, and sick sinus syndrome; however, no effective therapy exists. MOG1 increases plasma membrane (PM) expression of Na(v)1.5 and sodium current (I(Na)) density, thus w...
Topics
- Animals
- Brugada Syndrome
- Cells, Cultured
- DNA Mutational Analysis
- Disease Models, Animal
- Mice
- Mice, Knockout
- Mutation
- Myocytes, Cardiac
- NAV1.5 Voltage-Gated Sodium Channel
- RNA
- Sick Sinus Syndrome