Disease modeling of mosaic CINCA syndrome with iPS cells
Project/Area Number |
25461591
|
Research Category |
Grant-in-Aid for Scientific Research (C)
|
Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Pediatrics
|
Research Institution | Kyoto University |
Principal Investigator |
Saito Megumu 京都大学, iPS細胞研究所, 准教授 (90535486)
|
Project Period (FY) |
2013-04-01 – 2016-03-31
|
Project Status |
Completed (Fiscal Year 2015)
|
Budget Amount *help |
¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000)
Fiscal Year 2015: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
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)
|
Keywords | CINCA症候群 / 体細胞モザイク / iPS細胞 |
Outline of Final Research Achievements |
Taking advantage of iPS cells from CINCA syndrome patients with somatic mosaicism, we aimed at 1) exploring the pathogenic mechanism of somatic mosaicism, and investigating the function of compounds that might inhibit the activity of NLRP3 inflammasome.As a result, 1) our data suggested that the somatic ontogeny might be tracked using iPS cells, 2) we established a screening system using iPS cell-derived monocytic cells and monocytic cell lines.
|
Report
(4 results)
Research Products
(37 results)
-
-
-
-
[Journal Article] Modeling the early phenotype at the euromuscular junction of spinal muscular atrophy using patient-derived iPSCs.2015
Author(s)
Yoshida, M., Kitaoka, S., Egawa N., Yamane M., Ikeda R., Tsukita K., Amano N., Watanabe A., Morimoto, M., Takahashi J., Hosoi H., Nakahatan, T., Inoue, H.,Saito, Megumu K.
-
Journal Title
Stem Cell Reports
Volume: 4
Issue: 4
Pages: 1-8
DOI
Related Report
Peer Reviewed
-
[Journal Article] Pluripotent cell models of Fanconi anemia identify the early pathological defect in human hemoangiogenic progenitors.2015
Author(s)
Suzuki NM, Niwa A, Yabe M, Hira A, Okada C, Amano N, Watanabe A, Watanabe K, Heike T, Takata M, Nakahata T, Saito MK
-
Journal Title
Stem Cells Transl Med
Volume: 4
Issue: 4
Pages: 333-338
DOI
NAID
Related Report
Peer Reviewed / Open Access
-
[Journal Article] Enhanced chondrogenesis of induced pluripotent stem cells from patients with neonatal-onset multisystem inflammatory disease occurs via the caspase 1-independent cAMP/protein kinase A/CREB pathway2015
Author(s)
Yokoyama, K. Ikeya, M. Umeda, K. Oda, H. Nodomi, S. Nasu, A. Matsumoto, Y. Izawa, K. Horigome, K. Kusaka, T. Tanaka, T. Saito, M. K. Yasumi, T. Nishikomori, R. Ohara, O. Nakayama, N. Nakahata, T. Heike, T. Toguchida, J.
-
Journal Title
Arthritis Rheumatol
Volume: 67
Issue: 1
Pages: 302-314
DOI
Related Report
Peer Reviewed / Open Access / Acknowledgement Compliant
-
-
-
-
-
[Journal Article] Derivation of mesenchymal stromal cells from pluripotent stem cells through a neural crest lineage using small molecule compounds with defined media.2014
Author(s)
Fukuta M, Nakai Y, Kirino K, Nakagawa M, Sekiguchi K, Nagata S, Matsumoto Y, Yamamoto T, Umeda K, Heike T, Okumura N, Koizumi N, Sato T, Nakahata T, Saito M, Otsuka T, Kinoshita S, Ueno M, Ikeya M, Toguchida J.
-
Journal Title
PLOS ONE
Volume: 9
Issue: 12
Pages: e112291-e112291
DOI
Related Report
Peer Reviewed / Open Access / Acknowledgement Compliant
-
-
[Journal Article] Somatic NLRP3 mosaicism in Muckle-Wells syndrome. A genetic mechanism shared by different phenotypes of cryopyrin-associated periodic syndromes.2014
Author(s)
Nakagawa K, Kawai T, Umebayashi H, Takei S, Kobayashi N, Yashiro M, Kubota T, Koike R, Akuta N, Shimoyama K, Iwata N, Saito MK, Ohara O, Kambe N, Yasumi T, Izawa K, Kawai T, Heike T, Yagüe J, Nishikomori R, Aróstegui JI et al.
-
Journal Title
Ann Rheum Dis.
Volume: in press
Issue: 3
Pages: 1-8
DOI
Related Report
Peer Reviewed / Open Access
-
[Journal Article] HAX1 in induced pluripotent stem cells from a patient with severe congenital neutropenia improves defective granulopoiesis.2014
Author(s)
Morishima T, Watanabe K, Niwa A, Hirai H, Saida S, Tanaka T, Kato I, Umeda K, Hiramatsu H, Saito MK, Matsubara K, Adachi S, Kobayashi M, Nakahata T, Heike T
-
Journal Title
Haematologica
Volume: 99(1)
Issue: 1
Pages: 19-27
DOI
Related Report
Peer Reviewed / Open Access
-
[Journal Article] Robust and highly-efficient differentiation of functional monocytic cells from human pluripotent stem cells under serum- and feeder cell- free conditions.2013
Author(s)
Yanagimachi MD, Niwa A, Tanaka T, Ozaki F, Nishimoto S, Murata Y, Yasumi T, Ito J, Tomida S, Oshima K, Asaka I, Goto H, Heike T, Nakahata T, Saito MK
-
Journal Title
PLoS ONE
Volume: 8(4)
Issue: 4
Pages: e59243-e59243
DOI
NAID
Related Report
Peer Reviewed
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-