| Project/Area Number |
25670110
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| Research Category |
Grant-in-Aid for Challenging Exploratory Research
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| Allocation Type | Multi-year Fund |
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| Research Field |
General physiology
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| Research Institution | Tottori University |
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Principal Investigator |
HISATOME Ichiro 鳥取大学, 医学(系)研究科(研究院), 教授 (60211504)
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| Co-Investigator(Kenkyū-buntansha) |
SHIRAYOSHI Yasuaki 鳥取大学, 医学研究科, 准教授 (90249946)
IKEDA Nobuhito 鳥取大学, 医学研究科, 助教 (50620316)
MIAKE Junichiro 鳥取大学, 医学部, 講師 (40372677)
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| Project Period (FY) |
2013-04-01 – 2015-03-31
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| Project Status |
Completed (Fiscal Year 2014)
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| Budget Amount *help |
¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2014: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2013: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | 疾患iiPS / LQT1 / 熱ショック蛋白 / iPS cell / LQT / ion channel / 疾患iPS細胞 / HSP70 |
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| Outline of Final Research Achievements |
研究成果の概要（英文）：We identified the novel missense mutation M437V of KCNQ1 in a patient with QT interval prolongation (Long QT type 1: LQT1).We employed iPS cell (iPSC)-derived cardiomyocytes to investigate the electrophysiological properties of the mutant channel in LQT1 cardiomyocytes and to see effects of heat shock proteins. To generate iPSCs from the patient and a normal subject, peripheral blood T cells were reprogrammed. Differentiated cardiomyocytes from LQT1 iPSCs exhibited prolongation of action potential duration (APD), which was due to a reduction of the KCNQ1-mediated current IKs; Na+ and Ca2+ channel currents, as well as K+ channel currents other than IKs were comparable to those of cardiomyocytes from normal iPSCs and human ESCs. We tried to examine the heat shock, heat shock factor1 as well as heat shock protein 70 on the mutant channel properties of cardiomyocytes from LQT1 iPS cells.
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