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A Novel Humanized Neonatal Autoimmune Blistering Skin Disease Model Induced by Maternally Transferred Antibodies¹

Wataru Nishie^2,*, Daisuke Sawamura^*,, Ken Natsuga^*, Satoru Shinkuma^*, Maki Goto^*, Akihiko Shibaki^*, Hideyuki Ujiie^*, Edit Olasz, Kim B. Yancey and Hiroshi Shimizu^*

^* Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan; Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan; Department of Dermatology, Medical College of Wisconsin, Milwaukee, WI 53226; and Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, TX 75390

All mammal neonates receive maternal Abs for protection against pathogenic organisms in the postnatal environment. However, neonates can experience serious adverse reactions if the Abs transferred from the mother recognize self-molecules as autoAgs. In this study, we describe a novel model for autoimmune disease induced by transferred maternal Abs in genetically transformed Ag-humanized mice progeny. Bullous pemphigoid is the most common life-threatening autoimmune blistering skin disease that affects the elderly, in which circulating IgG autoAbs are directed against epidermal type XVII collagen (COL17). We have established a genetically manipulated experimental mouse model in which maternal Abs against human COL17 are transferred to pups whose skin expresses only human and not mouse COL17, resulting in blistering similar to that seen in patients with bullous pemphigoid. Maternal transfer of pathogenic Abs to humanized neonatal mice is a unique and potential experimental system to establish a novel autoimmune disease model.

The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked advertisement in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

¹ This work was supported in part by Grant-in-Aid for Young Scientists Start-up and Young Scientists A (19890005 and 20689021 to W.N.); by Grant-in-Aid for Exploratory Research (19659279 to H.S.); and by Health and Labour Science Research Grants for Research on Measures for Intractable Diseases, from the Ministry of Health, Labour, and Welfare of Japan (to H.S.) and by Program for Promotion of Fundemental Studies in Health Science of the National Institute of Biomedical Innovation (NIBIO; to H.S.).

² Address correspondence and reprint requests to Dr. Wataru Nishie, Department of Dermatology, Hokkaido University Graduate School of Medicine, N15 W7, Sapporo, Japan. E-mail address: nishie{at}med.hokudai.ac.jp

³ Abbreviations used in this paper: BP; boullous pemphigoid; COL17; type XVII collagen; NC; noncollagenous; Tg, transgenic; IIF, indirect immunofluorescence; DIF; direct immunofluorescence, GST; gulutathione S-transferase.

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The JI 2009 183: 3557-3558. [Full Text]