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Comment
. 2015 Jan;125(1):99-101.
doi: 10.1172/JCI80014. Epub 2014 Dec 15.

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

Arthur A M Wilde, Pieter G Postema
Comment

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

Arthur A M Wilde et al. J Clin Invest. 2015 Jan.

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.

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