Article
The dominant negative LQT2 mutation A561V reduces wild-type HERG expression.
The Journal of biological chemistry - 14 Apr 2000
Kagan A, Yu Z, Fishman G I, McDonald T V
Abstract excerpt
HERG(1) K(+) channel mutations are responsible for one form of dominantly inherited long QT syndrome (LQT). Some LQT mutations exert a dominant negative effect on wild-type current expression. To investigate mechanisms of dominant-negative behavior, we co-expressed wild-type HERG with the A561V mutant in mammalian cells. Transfection with various cDNA ratios produced HERG K(+) current densities that approached a...
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