کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
5649336 | 1407122 | 2017 | 33 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Experimental Laminin 332 Mucous Membrane Pemphigoid Critically Involves C5aR1 and Reflects Clinical and Immunopathological Characteristics of the Human Disease
دانلود مقاله + سفارش ترجمه
دانلود مقاله ISI انگلیسی
رایگان برای ایرانیان
کلمات کلیدی
موضوعات مرتبط
علوم پزشکی و سلامت
پزشکی و دندانپزشکی
امراض پوستی
پیش نمایش صفحه اول مقاله
![عکس صفحه اول مقاله: Experimental Laminin 332 Mucous Membrane Pemphigoid Critically Involves C5aR1 and Reflects Clinical and Immunopathological Characteristics of the Human Disease Experimental Laminin 332 Mucous Membrane Pemphigoid Critically Involves C5aR1 and Reflects Clinical and Immunopathological Characteristics of the Human Disease](/preview/png/5649336.png)
چکیده انگلیسی
Mucous membrane pemphigoid is an autoantibody-mediated disease predominantly affecting the oral cavity, pharynx, and conjunctiva. Conjunctival lesions may lead to impaired vision and, finally, blindness. About 25% of mucous membrane pemphigoid patients generate autoantibodies against the α3 chain of laminin 332 (LAMα3), a structural protein of epidermal/epithelial basement membranes. Here, we established a mouse model by the passive transfer of rabbit IgG against the murine homologs of two immunodominant fragments in adult C57BL/6 mice (mLAMα3). After repeated subcutaneous injections of anti-mLAMα3 IgG erosions and crusts occurred predominantly around the snout, eyes, and on ears. Conjunctival and oral/pharyngeal lesions with subepithelial splitting were found in 80% and 100% of mice, respectively. In contrast, disease development was abrogated in FcRγ chain-deficient mice and markedly reduced in C5aR1-deficient mice. Furthermore, wild-type mice injected with anti-mLAMα3 F(abâ²)2 were completely protected. Our findings suggest a crucial codominant role of FcRγ and complement activation of the anti-mLAMα3 IgG-induced mouse model of mucous membrane pemphigoid. This model will help further discover the pathomechanisms of this devastating disease. Furthermore, it may be of use to explore the effect of urgently needed more specific anti-inflammatory mediators on mucosal and skin lesions in autoantibody-mediated diseases.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of Investigative Dermatology - Volume 137, Issue 8, August 2017, Pages 1709-1718
Journal: Journal of Investigative Dermatology - Volume 137, Issue 8, August 2017, Pages 1709-1718
نویسندگان
Eva Nina Heppe, Sabrina Tofern, Franziska S. Schulze, Akira Ishiko, Atsushi Shimizu, Christian Sina, Detlef Zillikens, Jörg Köhl, Stephanie Goletz, Enno Schmidt,