Functional Analysis of iPSC-derived Myocytes from a Patient with Carnitine Palmitoyltransferase II Deficiency
Project/Area Number |
23791200
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Research Category |
Grant-in-Aid for Young Scientists (B)
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Allocation Type | Multi-year Fund |
Research Field |
Pediatrics
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Research Institution | Fukuoka University |
Principal Investigator |
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Project Period (FY) |
2011 – 2012
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Project Status |
Completed (Fiscal Year 2013)
|
Budget Amount *help |
¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2012: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Fiscal Year 2011: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
|
Keywords | CPTII欠損症 / iPS細胞 / 先天代謝異常症 / 横紋筋融解症 / ベザフィブラート |
Research Abstract |
We induced differentiation of CPT II-deficient hiPSCs into mature myocytes in a highly efficient and reproducible manner and recapitulated some aspects of the disease phenotypes of CPT II deficiency in the myocyte disease models. This approach addresses the challenges of modeling the abnormality of FAO in CPT II deficiency using iPSC technology and has the potential to revolutionize translational research in this field.
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Report
(4 results)
Research Products
(6 results)
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[Journal Article] Functional Analysis of iPSC-derived Myocytes from a Patient with Carnitine Palmitoyltransferase II Deficiency2014
Author(s)
Tetsuhiko Yasuno, Kenji Osafune, Hidetoshi Sakurai, Isao Asaka, Akihito Tanaka, Seiji Yamaguchi, Kenji Yamada, Hirofumi Hitomi, Sayaka Arai, Yuko Kurose, Yasuki Higaki, Mizuki Sudo, Souichi Ando, Hitoshi Nakashima, Takao Saito, Hidetoshi Kaneoka
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Journal Title
Biochem. and Biophys. Res. Commun
Volume: (in press)
Issue: 2
Pages: 175-181
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Monitoring and robust induction of nephrogenic intermediate mesoder m from human pluripotent stem cells2013
Author(s)
Shin-Ichi Mae, Akemi Shono, Fumihiko Shiota, Tetsuhiko Yasuno, Masatoshi Kajiwara, Nanaka Gotoda-Nishimura, Sayaka Arai, Aiko Sato-Otubo, Taro Toyoda, Kazutoshi Takahashi, Naoki Nakayama, Chad A. Cowan, Takashi Aoi, Seishi Ogawa, Andrew P. McMahon, Shinya Yamanaka, Kenji Osafune
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Journal Title
Nature Communications
Volume: 4:1367
Issue: 1
Pages: 1367-1367
DOI
Related Report
Peer Reviewed
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