2013 Fiscal Year Final Research Report
Comprehensive analysis of a molecular mechanism of hyper IgE-emia
| Project/Area Number |
25670478
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| Research Category |
Grant-in-Aid for Challenging Exploratory Research
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | The University of Tokushima |
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Principal Investigator |
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| Project Period (FY) |
2013-04-01 – 2014-03-31
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| Keywords | 高IgE血症 / アトピー性皮膚炎 |
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| Research Abstract |
Hyper IgE syndrome is a complex primary immunodeficiency characterized by atopic dermatitis associated with extremely high serum IgE levels and susceptibility to staphylococcal skin abscesses and pneumonia. Our recent studies have demonstrated that dominant negative mutations in the signal transducer and activator of transcription 3 (STAT3) gene result in the hyper IgE syndrome.
Although defective differentiation of Th17 cells may partly accounts for the susceptibility to staphylococcal skin abscesses and pneumonia, the pathogenesis of atopic manifestations in hyper IgE-emia remains to be elucidated. To investigate molecular pathogenesis of hyper IgE syndrome, we established knock-in model mice expressing dominant negative STAT3. In the mice, serum IgE levels were at least 100 times higher than littermate wild-type control mice. With these new model mice in our hand, we will investigate a molecular pathogenesis of hyper IgE-emia in hyper IgE syndrome.
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[Journal Article] The skin is an important bulwark of acquired immunity against intestinal helminthes2013
Author(s)
Obata-Ninomiya K, Ishiwata K, Tsutsui H, Nei Y, Yoshikawa S, Kawano Y, Minegishi Y, Ohta N, Watanabe N, Kanuka H, Karasuyama H
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Journal Title
J Exp Med
Volume: 210
Pages: 2583-2595
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[Journal Article] Boisson-Dupuis S, Pue1 A, Casanova JL, Morio T, Kobayashi M. Simple diagnosis of STAT1 gain-of-function alleles in patients with chronic mucocutaneous candidiasis2013
Author(s)
Mizoguchi Y, Tsumura M, Okada S, Hirata O, Minegishi S, Imai K, Hyakuna N, Muramatsu H, Kojima S, Ozaki Y, Imai T, Takeda S, Okazaki T, Ito T, Yasunaga S, Takihara Y, Bryant VL, Kong XF, Cypowyj S
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Journal Title
J Leukoc Biol
Volume: (in press)
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