Mechanistic Basis for Type 2 Long QT Syndrome Caused by KCNH2 Mutations that Disrupt Conserved Arginine Residues in the Voltage Sensor

A422T mutation in HERG potassium channel retained in ER is rescurable by pharmacologic or molecular chaperones

(2012) Jia Guo et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Changes in Channel Trafficking and Protein Stability Caused by LQT2 Mutations in the PAS Domain of the HERG Channel

(2012) Carol A. Harley et al.   PLoS One

Trafficking-deficient hERG K+ channels linked to long QT syndrome are regulated by a microtubule-dependent quality control compartment in the ER

(2011) Jennifer L. Smith et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Rescue of Aberrant Gating by a Genetically Encoded PAS (Per-Arnt-Sim) Domain in Several Long QT Syndrome Mutant HumanEther-á-go-go-related Gene Potassium Channels

(2011) Elena C. Gianulis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Simulation of the Undiseased Human Cardiac Ventricular Action Potential: Model Formulation and Experimental Validation

(2011) Thomas O'Hara et al.   PLoS Computational Biology

Properties of WT and mutant hERG K+ channels expressed in neonatal mouse cardiomyocytes

(2010) Eric C. Lin et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Genetic Testing for Long-QT Syndrome

(2009) Suraj Kapa et al.   CIRCULATION

Hsp40 Chaperones Promote Degradation of the hERG Potassium Channel

(2009) Valerie E. Walker et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cooperative Interactions Between R531 and Acidic Residues in the Voltage Sensing Module of hERG1 Channels

(2008) David R. Piper et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY