Referenced in: none

Automatically associated channels: Kv10.1 , Nav1.5

Title: Bringing home the bacon? The next step in cardiac sodium channelopathies.

Authors: Arthur A M Wilde, Pieter G Postema

Journal, date & volume: J. Clin. Invest., 2015 Jan , 125, 99-101

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/25500878

Abstract
Mutations in SCN5A, which encodes the α subunit of the major cardiac sodium channel Na(V)1.5, are associated with multiple cardiac arrhythmias, including Brugada syndrome. It is not clear why mutations in SCN5A result in such a variety of cardiac phenotypes, and introduction of analogous Scn5a mutations into small-animal models has not recapitulated alterations in cardiac physiology associated with human disease. In this issue of the JCI, Park and colleagues present a pig model of cardiac sodium channelopathy that was generated by introducing a human Brugada syndrome-associated SCN5A allele. This large-animal model exhibits many phenotypes seen in patients with SCN5A loss-of-function mutations and has the potential to provide important insight into sodium channelopathies.