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

January 11, 2013
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.

Explore further: Researchers use stem cells to show connection between neural cell disruption and Parkinson's disease

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

Related Stories

Researchers use stem cells to show connection between neural cell disruption and Parkinson's disease

October 18, 2012
(Medical Xpress)—A diverse team of biologists has shown using induced pluripotent stem cells (iPSCs) that a gene mutation that causes malformations in the structure of the nuclear envelope of neural cells, is associated ...

Hair-cell-derived patient-specific heart cells for disease modeling and drug screening

August 29, 2011
Hair follicle keratinocytes offer a simple and accessible route to generate patient-specific induced pluripotent stem cells, iPSCs, with minimum inconvenience for the patients, shows study presented at the ESC Congress 2011 ...

Recommended for you

Could aggressive blood pressure treatments lead to kidney damage?

July 18, 2017
Aggressive combination treatments for high blood pressure that are intended to protect the kidneys may actually be damaging the organs, new research from the University of Virginia School of Medicine suggests.

Quantifying effectiveness of treatment for irregular heartbeat

July 17, 2017
In a small proof-of-concept study, researchers at Johns Hopkins report a complex mathematical method to measure electrical communications within the heart can successfully predict the effectiveness of catheter ablation, the ...

Concerns over side effects of statins stopping stroke survivors taking medication

July 17, 2017
Negative media coverage of the side effects associated with taking statins, and patients' own experiences of taking the drugs, are among the reasons cited by stroke survivors and their carers for stopping taking potentially ...

Study discovers anticoagulant drugs are being prescribed against safety advice

July 17, 2017
A study by researchers at the University of Birmingham has shown that GPs are prescribing anticoagulants to patients with an irregular heartbeat against official safety advice.

Protein may protect against heart attack

July 14, 2017
DDK3 could be used as a new therapy to stop the build-up of fatty material inside the arteries

Heart study finds faulty link between biomarkers and clinical outcomes

July 14, 2017
Surrogate endpoints (biomarkers), which are routinely used in clinical research to test new drugs, should not be trusted as the ultimate measure to approve new health interventions in cardiovascular medicine, according to ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.