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Objective: The long QT syndrome, N629D HERG mutation, alters the pore selectivity signature sequence, GFGN to GFGD. Heterologous co-expression of N629D and the wildtype HERG resulted in a relative loss of the selectivity of K + over Na + , but its physiologic relevance has not been assessed in cardiac myocytes. Methods and results: Accordingly, N629D was overexpressed, via adenoviral...
Objectives: We hypothesized that exposure of N629D/wildtype channels to transient increases in [K + ] o could alter the conformation of the outer vestibule and thus reverse the disease phenotype. N629D is a recently described mutation of the HERG1 gene that causes familial long QT syndrome. This mutation alters the pore signature sequence resulting in loss of K + selectivity...
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