2022 Fiscal Year Final Research Report
The analysis of the variable region of the patients with bullous pemphigoid autoantibody
| Project/Area Number |
20K08684
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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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| Review Section |
Basic Section 53050:Dermatology-related
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| Research Institution | Hirosaki University |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
澤村 大輔 弘前大学, 医学研究科, 教授 (60196334)
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| Project Period (FY) |
2020-04-01 – 2023-03-31
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| Keywords | 水疱性類天疱瘡 / 自己免疫性水疱症 / BP / 抗BP180 NC16a抗体 / 抗基底膜抗体 / 自己抗体可変領域 |
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| Outline of Final Research Achievements |
Bullous pemphigoid (BP) is an autoimmune blistering disease (AIBD) characterized by subepidermal blisters and erosive lesions on the skin. Autoantibodies against the NC16a domain of BP180 in the epidermal basement membrane zone are produced in patients with BP. We use antiBP180-NC16a antibody titers as a marker of disease activity in outpatients with BP. We evaluated correlation to serum levels of antiBP180-NC16a antibodies and thymus and activation-regulated chemokine (TARC/CCL17). We studied the anti-BP180-type mucous membrane pemphigoid, epidermolysis bullosa acquisita, anti-laminin γ1 (p200) pemphigoid, and linear immunoglobulin A/immunoglobulin G bullous dermatosis. We studied the B-cell of BP patients by flow cytometry. We also examined autoantibodies to BPAG1e trigger experimental bullous pemphigoid in mice.
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| Free Research Field |
皮膚科学
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| Academic Significance and Societal Importance of the Research Achievements |
水疱性類天疱瘡（ＢＰ）は全身に表皮下水疱が多発する，最も頻度が高い自己免疫性水疱症であり，高齢者に好発する．本症患者の血清中には，皮膚の基底膜を認識する抗基底膜抗体が存在する．その自己抗体が認識する自己抗原として，BP230とBP180の類天疱瘡抗原が同定されたが1497アミノ酸からなるBP180のなかの非常に狭い領域，76アミノ酸からなるNC16aドメインを認識していることが解明されている．そこで，患者の抗BP180抗体の可変領域のアミノ酸配列を解析する研究を着想した．さらに，これが解明されれば，本症の自己抗体産生の発症機構の一端を明らかにできると考える．
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