Darouna Kattygnarath, Svetlana Maugenre, Nathalie Neyroud, Elise Balse, Carole Ichai, Isabelle Denjoy, Gilles Dilanian, Raphaël P Martins, Véronique Fressart, Myriam Berthet, Jean Jacques Schott, Antoine Leenhardt, Vincent Probst, Hervé Le Marec, Bernard Hainque, Alain Coulombe, Stéphane N Hatem, Pascale Guicheney
BACKGROUND: Brugada syndrome (BrS) is caused mainly by mutations in the SCN5A gene, which encodes the α-subunit of the cardiac sodium channel Na(v)1.5. However, ≈ 20% of probands have SCN5A mutations, suggesting the implication of other genes. MOG1 recently was described as a new partner of Na(v)1.5, playing a potential role in the regulation of its expression and trafficking. We investigated whether mutations in MOG1 could cause BrS. METHODS AND RESULTS: MOG1 was screened by direct sequencing in patients with BrS and idiopathic ventricular fibrillation...
June 2011: Circulation. Cardiovascular Genetics