experimental human cell and tissue models of pemphigus天疱疮的实验性的人类细胞和组织模型.pdfVIP

Hindawi Publishing Corporation Dermatology Research and Practice Volume 2010, Article ID 143871, 8 pages doi:10.1155/2010/143871 Review Article Experimental Human Cell and Tissue Models of Pemphigus Gerda van der Wier, Hendri H. Pas, and Marcel F. Jonkman Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, P.O. Box 30001, 9700 RB Groningen, The Netherlands Correspondence should be addressed to Gerda van der Wier, g.van.der.wier@derm.umcg.nl Received 29 December 2009; Accepted 18 March 2010 Academic Editor: Peter J. Koch Copyright © 2010 Gerda van der Wier et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Pemphigus is a chronic mucocutaneous autoimmune bullous disease that is characterized by loss of cell-cell contact in skin and/or mucous membranes. Past research has successfully identified desmosomes as immunological targets and has demonstrated that acantholysis is initiated through direct binding of IgG. The exact mechanisms of acantholysis, however, are still missing. Experimental model systems have contributed considerably to today’s knowledge and are still a favourite tool of research. In this paper we will describe to what extent human cell and tissue models represent the in vivo situation, for example, organ cultures of human skin, keratinocyte cultures, and human skin grafted on mice and, furthermore, how suitable they are to study the pathogenesis of pemphigus. Organ cultures closely mimic the architecture of the epidermis but are less suitable to answer posed