2010 Fiscal Year Final Research Report
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 |
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| Research Field |
Pediatrics
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| Research Institution | Kyoto University |
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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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| 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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