Researchers use iPSCs to define optimal treatment for managing life-threatening arrhythmias

ECG results from a patient with LQTS (bottom), which have a prolonged QT interval, are shown relative to normal ECG results (top). Researchers used iPSCs derived from a young patient with LQTS to determine a course of treatment that helped manage the patient's life-threatening arrhythmias. Credit: Adler, E. 2013. J. Gen. Physiol. doi:10.1085/jgp.201310958 (image adapted from ECG tracings supplied by Robert Pass).

Researchers used induced pluripotent stem cells (iPSCs) derived from a young patient with Long QT syndrome (LQTS), a congenital heart disorder, to determine a course of treatment that helped manage the patient's life-threatening arrhythmias. The results, which appear in The Journal of General Physiology, could lead to improved treatments for LQTS and other channelopathies, diseases caused by disturbed ion channel function.

iPSCs— that have been genetically reprogrammed to function like —provide a valuable tool for studying diseases and developing customized drug therapies. Researchers from Columbia University Medical Center used iPSCs differentiated into cardiomyocytes (iPSCs-CMs) to study the physiological basis for arrhythmias in a four-year-old with LQTS.

The disease, which can cause arrhythmias that lead to seizures and sudden death, is caused by a mutation in any of various genes encoding cardiac ion channels or their associated proteins. In the case at hand, the child had mutation in the SCN5A gene, which encodes a sodium channel, and a common polymorphism in the KCNH2 gene, which encodes a potassium channel.

Using voltage clamp analyses of iPSCs-CMs derived from the affected child and his parents, the researchers determined that his arrhythmias were caused by the SCN5A mutation. They performed further in vitro testing using the iPSCs-CMs to identify the most appropriate regimen for correcting the aberrant activity associated with the defective ion channel.

The results show promise for using in vitro iPSC techniques in the development of individualized drug therapies for patients with LQTS and other channelopathies.

More information: Terrenoire, C., et al. 2013. J. Gen. Physiol. 141:61-72.

add to favorites email to friend print save as pdf

Related Stories

Induced pluripotent stem cells at risk for rejection

May 13, 2011

(PhysOrg.com) -- Biologists at UC San Diego have discovered that an important class of stem cells known as "induced pluripotent stem cells," or iPSCs, derived from an individual's own cells, could face immune ...

Recommended for you

An autoimmune response may contribute to hypertension

19 hours ago

High blood pressure is a major risk factor for heart attack, stroke, chronic heart failure, and kidney disease. Inflammation is thought to promote the development of high blood pressure, though it is not clear what triggers ...

Results of RIBS IV trial reported

Sep 16, 2014

A new clinical trial comparing the use of everolimus-eluting stents (EES) and drug-eluting balloons (DEB) in treating in-stent restenosis (ISR) from drug-eluting stents found that EES provided superior late angiographic results ...

Results of DKCRUSH-VI trial reported

Sep 16, 2014

A new study found that fractional flow reserve (FFR)-guided provisional side branch (SB) stenting of true coronary bifurcation lesions yields similar outcomes to the current standard of care. The DKCRUSH-VI clinical trial ...

User comments