Recently published data revealed a reduced NaV 1.5 current through defective functional NaV 1.5—ß-2-Syntrophin (SNTB2) interaction in native cardiomyocytes of BS patients associated with SNTB2 mutation emphasizing the role of SNTB2 in BS pathophysiology [25]. The gene discussed is SNTB1; the disease is Bloom syndrome.