Models for QT prolongation

A group at the Technion Israel Institute of Technology and a team at the Stanford University School of Medicinehave independently developed human stem cell-based models of long QT syndrome,^1,2 a potentially fatal cardiac disorder that can be triggered by environmental and pharmacological stimuli in susceptible patients. The models could be used to identify potential therapies for long QT syndrome as well as compounds that induce QT prolongation as a side effect-a problem that has derailed many drug development programs.

Of the 12 subtypes of long QT syndrome, the two most common-LQT1 and LQT2-are caused by mutations in potassium channel proteins that normallyhelp maintain the regular electrical activity of the heart. Other long QT subtypes are associated with mutations in additional proteins, including calcium channels, sodium channels and ankyrin 2 (ANK2)...