Establishing an innovative platform for the analysis of hemophagocytic lymphohistiocytosis
| Project/Area Number |
26461582
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | Kyoto University |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
HEIKE Toshio 京都大学, 大学院医学研究科発達小児科学, 教授 (90190173)
OHARA Osamu 公益財団法人かずさDNA研究所, ヒトゲノム研究部, 部長 (20370926)
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| Research Collaborator |
NISHIKOMORI Ryuta 京都大学, 大学院医学研究科 発達小児科学, 准教授 (70359800)
HORI Masayuki 京都大学, 大学院医学研究科 発達小児科学, 大学院生
HIEJIMA Eitaro 京都大学, 大学院医学研究科 発達小児科学, 大学院生
SHIBATA Hirofumi 京都大学, 大学院医学研究科 発達小児科学, 大学院生
SHIMODERA Saeko 京都大学, 大学院医学研究科 発達小児科学, 大学院生
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| Project Period (FY) |
2014-04-01 – 2017-03-31
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| Project Status |
Completed (Fiscal Year 2016)
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| Budget Amount *help |
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2015: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2014: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | 血球貪食性リンパ組織球症 / 迅速診断法 / 分子病態解析 / 機能解析 / 迅速診断 / 機能解析系の確立 / 細胞ライブラリー / バイオマーカー検索 / 迅速スクリーニング / 病態解析 / 機能病態解析 |
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| Outline of Final Research Achievements |
During the process of establishing a comprehensive screening system for familial hemophagocytic lymphohistiocytosis, we found that a CD57+ CTL degranulation assay more effectively identifies FHL3 patients than the NK cell-based assays.
We established CTL lines derived from FHL2 and FHL3 patients and immortalized some of these cells using herpesvirus saimiri. By transiently expressing PRF1 or UNC13D cDNA constructs into the established FHL model CTL lines, we succeeded in evaluating the effect of a given gene mutation on the protein expression, degranulation, and cytotoxic function.
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Report
(4 results)
Research Products
(9 results)
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[Journal Article] A CD57+ CTL degranulation assay effectively identifies familial hemophagocytic lymphohistiocytosis type 3 patients2017
Author(s)
Masayuki Hori, Takahiro Yasumi, Saeko Shimodera, Hirofumi Shibata, Eitaro Hiejima, Hirotsugu Oda, Kazushi Izawa, Tomoki Kawai, Masataka Ishimura, Naoko Nakano, Ryutaro Shirakawa, Ryuta Nishikomori, Hidetoshi Takada, Satoshi Morita, Hisanori Horiuchi, Osamu Ohara, Eiichi Ishii, and Toshio Heike
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Journal Title
Journal of Clinical Immunology
Volume: 37
Issue: 1
Pages: 92-99
DOI
Related Report
Peer Reviewed / Acknowledgement Compliant
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[Journal Article] Laboratory parameters identify familial haemophagocytic lymphohistiocytosis from other forms of paediatric haemophagocytosis2015
Author(s)
Yasumi T, Hori M, Hiejima E, Shibata H, Izawa K, Oda H, Yoshioka K, Nakagawa K, Kawai T, Nishikomori R, Ohara O, Heike T
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Journal Title
British Journal of Haematology
Volume: 170
Issue: 4
Pages: 532-538
DOI
Related Report
Peer Reviewed / Acknowledgement Compliant
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