Research: Patient-Specific Stem-Cell Models for Long-QT Syndrome September 3, 2010
Posted by Peng-Sheng Chen, MD, FHRS in Science & Research.Tags: Long-QT syndrome type 1, Tachyarrhythmia
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Patient-Specific Stem-Cell Models for Long-QT Syndrome
Moretti et al (NEJM 2010; Epub, PMID: 20660394) screened a family affected by long-QT syndrome type 1 (LQT1) and identified an autosomal dominant missense mutation (R190Q) in the KCNQ1 gene.
They infected dermal fibroblasts from family members and healthy controls with retroviral vectors to generate pluripotent stem cells, which were then directed to differentiate into cardiac myocytes. The action potential duration was markedly prolonged in cells derived from patients with LQT1, as compared with cells from control subjects. The LQT1 myocytes had an increased susceptibility to catecholamine-induced tachyarrhythmia and that beta-blockade attenuated this phenotype.
The authors conclude that they generated patient-specific pluripotent stem cells from family members affected by LQT1 and induced them to differentiate into functional cardiac myocytes. The patient-derived cells recapitulated the electrophysiological features of the disorder.
Heart Rhythm Society
Laurie Racenet, RN, MSN, ANP
Michael Giudici, MD, FACC, FACP, FHRS
Heather M. Ross, DNP, ANP-BC, CCDS, CEPS, FHRS
Anne Dubin, MD, FHRS
John D. Day, MD, FHRS
Andrea M. Russo, MD, FACC, FHRS
Joshua M. Cooper, MD, FACC
Amit J. Shanker, MD, FHRS, FACC
Bruce Stambler, MD
David Wiggins, RN, BS BME, CEPS
Fred M. Kusumoto, MD, FHRS
Leonard Ganz, MD, FHRS
christinechiuman
Craig Delaughter, MD, PhD
Ariana Del Negro, Heart Rhythm 2010 Quality
Peng-Sheng Chen, MD, FHRS
MSKremers MD,FHRS
Ann C. Garlitski, MD, FACC, FHRS
Alan Cheng, MD, FHRS
Ulrika Birgersdotter-Green, MD
Christopher Clyne, MD, FHRS
Kathleen Blake, MD, MPH, FHRS
David Scher, MD, FHRS
Sanjay Deshpande, MD, FHRS
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