EP News: Basic and Translational

KCNQ1 is the pore-forming subunit of cardiac slow-delayed rectifier potassium channels. Mutations in the kcnq1 gene are the leading cause of congenital long QT syndrome (LQTS). Sun and MacKinnon (Cell 2017;169:1042, PMID 28575668) presented the cryoelectron microscopy structure of a KCNQ1/calmodulin (CaM) complex. The conformation corresponds to an “uncoupled” KCNQ1, with activated voltage sensors and a closed pore. Unique structural features within the S4-S5 linker permit uncoupling of the voltage sensor from the pore in the absence of PIP2.
Source: Heart Rhythm - Category: Cardiology Authors: Source Type: research