A novel humanized neonatal autoimmune blistering skin disease model induced by maternally transferred antibodies

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

Research output: Contribution to journalArticle

21 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)4088-4093
Number of pages6
JournalJournal of Immunology
Volume183
Issue number6
DOIs
StatePublished - Sep 15 2009

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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    Nishie, W., Sawamura, D., Natsuga, K., Shinkuma, S., Goto, M., Shibaki, A., Ujiie, H., Olasz, E., Yancey, K. B., & Shimizu, H. (2009). A novel humanized neonatal autoimmune blistering skin disease model induced by maternally transferred antibodies. Journal of Immunology, 183(6), 4088-4093. https://doi.org/10.4049/jimmunol.0800389