2012 Fiscal Year Final Research Report
Generation of iPS cells derived form an Rett syndrome model mouse and drug screening for Rett syndrome using MeCP2 deficient iPS cells
| Project/Area Number |
22591144
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | Kurume University |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
MATSUISHI Toyojiro 久留米大学, 医学部, 教授 (60157237)
TANAKA Eiichiro 久留米大学, 医学部, 教授 (80188284)
MURAI Yoshinaka 久留米大学, 医学部, 准教授 (40322820)
NISHI Yoshihiro 久留米大学, 医学部, 講師 (20352122)
OKABE Yasunori 久留米大学, 医学部, 助教 (00446098)
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| Project Period (FY) |
2010 – 2012
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| Keywords | Rett 症候群 |
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| Research Abstract |
Rett syndrome (RTT) is a neurodevelopmetal disorder associated with mutations in the methyl-CpG?binding protein 2 (MeCP2) gene. MeCP2-deficient mice recapitulate the neurological degeneration observed in RTT patients. The generation of diseased induced pluripotetn stem (iPS) cell is a critical step in understanding the pathogenesis of genetics and complex diseases. Here, we generated RTT-model mouse iPS cell lines from MeCP2-deficient mouse by co-expressing four factors (Oct3/4, Sox2, Klf4, and c-Myc) throughlentiviraldeliveryandsubsequentlydifferentiatedthemintoneuronalcellsfor further analyses. RTT-model iPS cells were characterized for the expression of pluripotent markers and for their ability to differentiate into all three germ layers by forming embryoid bodies in vitro. In addition, SDIA-induced colonies from both WT and RTT-model iPS cell lines generated cells possessing neuronal-like morphologies that expressed neuronal markers microtubule-associated protein 2 (MAP2) an neuron-specific tubulin beta type III. Our RTT-model iPS cell lines provide a powerful tool for both basic neuroscience research and therapeutic applications.
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[Journal Article] Two neonatal cholestsis patients with mutations in the SRD5B1 (AKR1D1) gene: diagnosis and bile acid profiles during chenodeoxycholic acid treatment.2013
Author(s)
Seki, Y., Mizuochi, T., Kimura, A., Takahashi T., Ohtake, A., Hayashi, S., Morimura, T., Ohno, Y., Hoshina, T., Ihara, K., Takei, H., Nittono H., Kurosawa T., Homma K., Hasegawa T., Matsuishi T.
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Journal Title
J Inherit Metab Dis
Volume: 36(3)
Pages: 565-573
DOI
Peer Reviewed
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[Journal Article] Characterization of urinary bile acids in a pediatric BRIC-1 patient: Effectof rifampicin treatment.2012
Author(s)
Mizuochi T., Kimura A., Tanaka A., Muto A., Nittono H., Seki Y., Takahashi T., Kurosawa T., Kage M., Takikawa H., Matsuishi T.
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Journal Title
Clin Chim Acta
Volume: 413(15-16)
Pages: 1301-1304
DOI
Peer Reviewed
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[Journal Article] Molecular genetics and bile acid profiles of 3beta-hydroxy-delta5-C27-steroid dehydrogenase/isomerase deficiency in 2 Japanise patients.2010
Author(s)
Mizuochi T., Kimura A., Ueki I., Takahashi T., Hashimoto T., Takao A., Seki Y., Takei H., Nittono H., Kurosawa T., Matsuishi T.
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Journal Title
Pediatr Res
Volume: 68(3)
Pages: 258-263
DOI
Peer Reviewed
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[Journal Article] Minatoguchi S.Anti-Fas Gene Therapy Prevents Doxorubicin-induced Cardiotoxicity through Mechanisms Independent ofApoptosis.2010
Author(s)
Miyata S., Takemura G., Kosai K., Takahashi T., Esaki M., Li L., Kanamori H., Maruyama R., Goto K., Tsujimoto A., Takeyama T, Kawaguchi T., Ohno T., Nishigaki K., Fujiwara T., Fujiwara H.
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Journal Title
Am J Pathol
Volume: 176(2)
Pages: 687-698
DOI
Peer Reviewed
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[Presentation] Involvement of myeloid dendritic cell in the pathogenesis of acute phase of Kawasaki disease.2010
Author(s)
SudaK,Kishimoto S, Takahashi T,Okamura H, Yasukawa H, Nishino H, Teramachi Y, Itoh S, Yokoyama T, Iemura M, Imaizumi T, MatsushiT.
Organizer
The 74th annual scientific meeting of the Japanese Circulation Society
Place of Presentation
Kyoto,Japan
Year and Date
20100305-0307
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