Analysos of metabolic abnormality which causes clonal expansion in Paroxysmal nocturnal hemoglobinuria
| Project/Area Number |
15K15362
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| Research Category |
Grant-in-Aid for Challenging Exploratory Research
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| Allocation Type | Multi-year Fund |
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| Research Field |
Hematology
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| Research Institution | Osaka University |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
Kinoshita Taroh 大阪大学, 微生物病研究所, 教授 (10153165)
Inoue Norimitsu 大阪府立成人病センター, 部門長 (80252708)
Nishimura Junichi 大阪大学, 医学部, 助教 (80464246)
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| Project Period (FY) |
2015-04-01 – 2017-03-31
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| Project Status |
Completed (Fiscal Year 2016)
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| Budget Amount *help |
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2016: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2015: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
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| Keywords | アルカリホスファターゼ / ビタミンB１ / ビタミンB６ / 発作性夜間ヘモグロビン尿症 / GPI アンカー / clonal expansion / PIGA / ビタミンB6 / GPI / PNH / ビタミンB1 |
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| Outline of Final Research Achievements |
Paroxysmal Nocturnal hemoglobinuria (PNH) is the hematopoietic disease caused by the somatic mutation of PIGA gene. PIGA is essential enzyme for biosynthesis of GPI anchor, which anchor the various GPI anchored protein to the cell surface. So, PIGA deficient cells will be GPI negative cells. These GPI negative cells expand, however, the mechanism of which is unknown. Alkaline phosphatase (ALP) is one of the GPI-anchored proteins and it is defective in GPI negative cells. ALP is involved in taking up the vitamins into the cells and defect in ALP causes deficiency of vitamins in the cells, leading to various metabolic abnormalities. We speculate this causes clonal expansion of GPI negative cells. Analysis of concentration of vitamins in the cells or serum of the model mice indicated that mutant mice showed decrease in taking up vitamins.
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Report
(3 results)
Research Products
(5 results)
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[Presentation] Paroxysmal nocturnal hemoglobinuria caused by PIGT mutations; Atypical PNH2016
Author(s)
Yoshiko Murakami, Michi Kawamoto, Norimitsu Inoue, Makiko Osato, Shogo Murata, Sho Murase, Hajime Yoshimura, Yasutaka Ueda, Jun-ichi Nishimura, Yuzuru Kanakura, Nobuo Kohara, and Taroh Kinoshita
Organizer
The 58th ASH Annual Meeting
Place of Presentation
San Diego
Year and Date
2016-12-04
Related Report
Int'l Joint Research
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