The C5a Receptor on Mast Cells Is Critical for the Autoimmune Skin-blistering Disease Bullous Pemphigoid

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The Autoimmune Skin Disease Bullous Pemphigoid: The Role of Mast Cells in Autoantibody-Induced Tissue Injury

Bullous pemphigoid (BP) is an autoimmune and inflammatory skin disease associated with subepidermal blistering and autoantibodies directed against the hemidesmosomal components BP180 and BP230. Animal models of BP were developed by passively transferring anti-BP180 IgG into mice, which recapitulates the key features of human BP. By using these in vivo model systems, key cellular and molecular e...

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Doxycycline-Inducible Autoimmune Blistering Skin Disease Model

W. Nishie , M.D., Ph.D. • H. Shimizu , M.D., Ph.D. (*) Department of Dermatology , Hokkaido University Graduate School of Medicine , N15W7, Kita-ku , Sapporo 003-0835 , Japan e-mail: [email protected] Abstract Bullous pemphigoid (BP) is the most common autoimmune blistering disorder. BP autoantibodies target two hemidesmosomal components, collagen XVII (COL17) and BP230, with autoimmuni...

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BP180 Is Critical in the Autoimmunity of Bullous Pemphigoid

Bullous pemphigoid (BP) is by far the most common autoimmune blistering dermatosis that mainly occurs in the elderly. The BP180 is a transmembrane glycoprotein, which is highly immunodominant in BP. The structure and location of BP180 indicate that it is a significant autoantigen and plays a key role in blister formation. Autoantibodies from BP patients react with BP180, which leads to its degr...

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Mediators of Mast Cells in Bullous Pemphigoid and Dermatitis Herpetiformis

Bullous pemphigoid (BP) and dermatitis herpetiformis (DH) are skin diseases associated with inflammation. However, few findings exist concerning the role of mast cells in autoimmune blistering disease. Skin biopsies were taken from 27 BP and 14 DH patients, as well as 20 healthy individuals. Immunohistochemistry was used to identify the localization and mast cell expression of TNFα and MMP9 in ...

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The Role of Mast Cells in Autoimmune Bullous Dermatoses

Skin mast cells (MCs), a resident immune cell type with broad regulatory capacity, play an important role in sensing danger signals as well as in the control of the local immune response. It is conceivable to expect that skin MCs regulate autoimmune response and are thus involved in autoimmune diseases in the skin, e.g., autoimmune bullous dermatoses (AIBD). Therefore, exploring the role of MCs...

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ژورنال

عنوان ژورنال: Journal of Biological Chemistry

سال: 2011

ISSN: 0021-9258

DOI: 10.1074/jbc.m111.221036