title: Bringing home the bacon? The next step in cardiac sodium channelopathies.
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.
authors.forename: Arthur A M
authors.initials: AA
authors.lastname: Wilde
authors.forename: Pieter G
authors.initials: PG
authors.lastname: Postema
mesh_terms: D000818:Animals; D001145:Arrhythmias, Cardiac / Q000235:genetics*; D053840:Brugada Syndrome / Q000235:genetics*; D000075224:Cardiac Conduction System Disease; D006329:Heart Conduction System / Q000002:abnormalities*; D006801:Humans; D062554:NAV1.5 Voltage-Gated Sodium Channel / Q000235:genetics*
chemical_list: D062554:NAV1.5 Voltage-Gated Sodium Channel