Potassium Channel Subunits: The MiRP Family

Mutations of the MiRP1-encoding geneKCNE2 can severely compromise VGK currents and are associated with arrhythmia. The schematic of MiRP1 indicates disease-associated mutations. Residues highlighted in red represent missense mutations (i.e., Q9E, M54T, I57T, and A116V) that have been functionally characterized and clinically associated with arrhythmia (see text for details); the residue highlighted in blue represents the site of a polymorphism (i.e., T8A) associated with drug-induced arrhythmia (CHO, predicted sites of glycosylation; P, predicted sites of phosphorylation).