| Project/Area Number |
23591661
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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 |
Dermatology
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| Research Institution | Hyogo Medical University |
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Principal Investigator |
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| Research Collaborator |
IMAI Yasutomo 兵庫医科大学, 医学部, 講師 (10529514)
NAKAGAWA Noboru 兵庫医科大学, 医学部, 講師 (90412014)
YAMAMOTO Masaaki 兵庫医科大学, 医学部, 講師
SAKAGUCHI Yoshiko 兵庫医科大学, 実験補助
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| Project Period (FY) |
2011 – 2013
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| Project Status |
Completed (Fiscal Year 2013)
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| Budget Amount *help |
¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2013: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2012: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2011: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | 角化症 / 魚鱗癬 / 病態 / モデル動物 / サイトカイン / 魚鱗癬様紅皮症 / ネザートン症候群 / ケラチン / トランスグルタミナーゼ / 常染色体劣性魚鱗癬 / トランスグルタミナーゼ１ / 温度感受性 / 葉状魚鱗癬 |
|---|
| Research Abstract |
Various phenotypes and pathophysiology were examined in Causative mutations in congenital ichthyoses including rare cases with epidermolytic ichthyosis with hypercalcemia and bathing suit ichthyosis with summer exacerbation. A knock-in mouse line with a transglutaminase 1 mutation R142C was generated using Cre-lox system and mice were subjected for analysis of the phenotype. Homozygous mutant mice were lethal soon after birth and showed a low expression of the enzyme. Intercellular lipid organization in the stratum corneum was totally disoriented in living mutant mice. In cases with Netherton syndrome, which show atopic dermatitis-like phenotypes, the up-regulation of interleukin 33 was evident. IL-33 may be involved in the atopy-like cutaneous inflammation in Netherton syndrome, because a skin-specific expression of IL-33 in mice showed immunological features resembling atopic dermatitis.
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