2011 Fiscal Year Final Research Report
Study of innate immunity in primary antibody deficiency
| Project/Area Number |
21591350
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | University of Toyama |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
ISHIKAWA Fumihiko 理化学研究所, 免疫アレルギー科学総合研究センター ヒト疾患モデル研究ユニット, ユニットリーダー (30403918)
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| Project Period (FY) |
2009 – 2011
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| Keywords | 抗体産生不全症 / 自然免疫 / X連鎖無ガンマグロブリン血症 / Toll様受容体 / ブルトンチロシンキナーゼ |
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| Research Abstract |
Approximately 15-20% of patients with X-linked agammaglobulinemia(XLA), which is caused by BTK deficiency, have neutropenia, and it is associated with the severity of infection. BTK-deficient neutrophils had more production of reactive oxygen species(ROS) after various stimuli, which was associated with more apoptosis. The production of ROS and apoptosis of BTK-deficient neutrophils were reversed by transduction of recombinant BTK. These data suggest that severe neutropenia in patients with XLA can be treated with recombinant BTK.
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