Heteromerization of Kir2.x potassium channels contributes to the phenotype of Andersen's syndrome

摘要

Andersen's syndrome, an autosomal dominant disorder related to mutations of the potassium channel Kir2.1, is characterized by cardiac arrhythmias, periodic paralysis, and dysmorphic bone struc- ture. The aim of our study was to find out whether heteromeriza- tion of Kir2.1 channels with wild-type Kir2.2 and Kir2.3 channels contributes to the phenotype of Andersen's syndrome. The fol- lowing results show that Kir2.x channels can form functional heteromers: (i) HEK293 cells transfected with Kir2.x-Kir2.y con- catemers expressed inwardly rectifying K~+ channels with a con- ductance of 28-30 pS.