Modeling and analyzing pathogenesis of cryopyrin-associated periodic syndrome using induced pluripotent stem cells
Project/Area Number |
21890118
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Research Category |
Grant-in-Aid for Research Activity Start-up
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Allocation Type | Single-year Grants |
Research Field |
Pediatrics
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Research Institution | Kyoto University |
Principal Investigator |
SAITO Megmu Kyoto University, iPS細胞研究所, 特定拠点助教 (90535486)
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Project Period (FY) |
2009 – 2010
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Project Status |
Completed (Fiscal Year 2010)
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Budget Amount *help |
¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000)
Fiscal Year 2010: ¥1,235,000 (Direct Cost: ¥950,000、Indirect Cost: ¥285,000)
Fiscal Year 2009: ¥1,365,000 (Direct Cost: ¥1,050,000、Indirect Cost: ¥315,000)
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Keywords | Cryopyrin関連周期熱症候群 / NLRP3 / 疾患iPS細胞 / iPS細胞 / 血球分化 |
Research Abstract |
Cryopyrin-associated periodic syndrome (CAPS) is an severe autoinflammatory syndrome caused by mutations of NLRP3. To investigate the mechanism of disease and for drug screening, we established patient-specific induced pluripotent stem (iPS) cells from two CAPS patients with somatic mosaicism. Both mutant and wild type clones were obtained, and these clones were differentiated into macrophages. Disease-associated features such as overproduction of IL-1beta, pyronecrosis were observed only in mutant clones. Thus we succeeded to establish a disease-model of CAPS using iPS cells.
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Report
(3 results)
Research Products
(29 results)
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[Journal Article] Role of the NOD2 genotype in the clinical phenotype of Blau syndrome and early-onset sarcoidosis2009
Author(s)
Okafuji I, Nishikomori R, Kanazawa N, Kambe N, Fujisawa A, Yamazaki S, Saito M, Yoshioka T, Kawai T, Sakai H, Tanizaki H, Heike T, Miyachi Y, Nakahata T.
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Journal Title
Arthritis Rheum. 60
Pages: 242-250
Related Report
Peer Reviewed
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