2014 Fiscal Year Final Research Report
Modeling and analyzing of long QT syndrome using disease-specific iPS cells
| Project/Area Number |
25870331
|
|---|
| Research Category |
Grant-in-Aid for Young Scientists (B)
|
| Allocation Type | Multi-year Fund |
|---|
| Research Field |
Cardiovascular medicine
General physiology
|
|---|
| Research Institution | Shiga University of Medical Science |
|---|
Principal Investigator |
|
|---|
| Co-Investigator(Renkei-kenkyūsha) |
KURODA Yusuke 名古屋大学, 医学部
YUASA Shinsuke 慶應義塾大学, 医学部, 講師
|
|---|
| Project Period (FY) |
2013-04-01 – 2015-03-31
|
| Keywords | 不整脈 / 分子心臓病態学 |
|---|
| Outline of Final Research Achievements |
Long QT syndrome type 7 (LQT-7) is caused by a mutation in the KCNJ2 gene, and characterized by ventricular tachyarrhythmias associated with QT/QU interval prolongation, periodic paralysis and dysmorphic features. We generated induced pluripotent stem cells (iPSCs) from three LQT-7 patients carrying the KCNJ2 different mutations, and analyzed iPSC-derived cardiomyocytes (iPS-CMs).We measured action potentials in single iPS-CMs. There are no significant difference in action potential duration at 50% and 90% repolarization, and maximum diastolic potential between LQT-7 and wild type. In multi-electrode arrays analyses, flecainide reduced ectopic activities in LQT-7-derived cardiomyocytes. In condition with flecainide pre-administration, isoproterenol did not induce arrhythmic events, which suggests that flecainide has prophylactic effect in LQT-7-derived cardiomyocytes.
This study suggest that iPS-CM could be a useful model for exploring disease mechanisms and drug screening.
|
| Free Research Field |
循環器内科学
|
