[HTML][HTML] The dominant negative LQT2 mutation A561V reduces wild-type HERG expression
A Kagan, Z Yu, GI Fishman, TV McDonald - Journal of Biological Chemistry, 2000 - ASBMB
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 predicted binomial
distribution where mutant and wild-type subunits co-assemble in a tetramer with nearly …
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 predicted binomial
distribution where mutant and wild-type subunits co-assemble in a tetramer with nearly …
