[HTML][HTML] Thapsigargin selectively rescues the trafficking defective LQT2 channels G601S and F805C
BP Delisle, CL Anderson, RC Balijepalli… - Journal of Biological …, 2003 - ASBMB
Several mutations in the human ether-a-go-go-related K+ channel gene (HERG or KCNH2)
cause long QT syndrome (LQT2) by reducing the intracellular transport (trafficking) of the
channel protein to the cell surface. Drugs that bind to and block HERG channels (ie E4031)
rescue the surface expression of some trafficking defective LQT2 mutations. Because these
drugs potently block HERG current, their ability to correct congenital LQT is confounded by
their risk of causing acquired LQT. We tested the hypothesis that pharmacological rescue …
cause long QT syndrome (LQT2) by reducing the intracellular transport (trafficking) of the
channel protein to the cell surface. Drugs that bind to and block HERG channels (ie E4031)
rescue the surface expression of some trafficking defective LQT2 mutations. Because these
drugs potently block HERG current, their ability to correct congenital LQT is confounded by
their risk of causing acquired LQT. We tested the hypothesis that pharmacological rescue …