[HTML][HTML] The binding site for channel blockers that rescue misprocessed human long QT syndrome type 2 ether-a-gogo-related gene (HERG) mutations

E Ficker, S Zhao, CA Obejero-Paz, AM Brown - Journal of Biological …, 2002 - ASBMB
Mutations in the humanether-a-gogo-related gene (HERG) K+ channel gene cause
chromosome 7-linked long QT syndrome type 2 (LQT2), which is characterized by a
prolonged QT interval in the electrocardiogram and an increased susceptibility to life-
threatening cardiac arrhythmias. LQT2 mutations produce loss-of-function phenotypes and
reduceI Kr currents either by the heteromeric assembly of non-or malfunctioning channel
subunits with wild type subunits at the cell surface or by retention of misprocessed mutant …