title: A novel humanized neonatal autoimmune blistering skin disease model induced by maternally transferred antibodies.
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.
authors.affiliation: Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan. nishie@med.hokudai.ac.jp
authors.forename: Wataru
authors.initials: W
authors.lastname: Nishie
authors.forename: Daisuke
authors.initials: D
authors.lastname: Sawamura
authors.forename: Ken
authors.initials: K
authors.lastname: Natsuga
authors.forename: Satoru
authors.initials: S
authors.lastname: Shinkuma
authors.forename: Maki
authors.initials: M
authors.lastname: Goto
authors.forename: Akihiko
authors.initials: A
authors.lastname: Shibaki
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Ujiie
authors.forename: Edit
authors.initials: E
authors.lastname: Olasz
authors.forename: Kim B
authors.initials: KB
authors.lastname: Yancey
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shimizu
mesh_terms: D000818:Animals; D000831:Animals, Newborn; D001323:Autoantibodies; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases; D004195:Disease Models, Animal; D005260:Female; D006801:Humans; D008431:Maternal-Fetal Exchange / Q000276:immunology*; D051379:Mice; D024041:Non-Fibrillar Collagens / Q000276:immunology; D010391:Pemphigoid, Bullous / Q000276:immunology*; D011247:Pregnancy; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII