Faculty Collaboration Database

A novel humanized neonatal autoimmune blistering skin disease model induced by maternally transferred antibodies. J Immunol 2009 Sep 15;183(6):4088-93

Date

09/01/2009

Pubmed ID

19717520

DOI

10.4049/jimmunol.0800389

Scopus ID

2-s2.0-70349321267 (requires institutional sign-in at Scopus site)   25 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.

Author List

Nishie W, Sawamura D, Natsuga K, Shinkuma S, Goto M, Shibaki A, Ujiie H, Olasz E, Yancey KB, Shimizu H

Author

Edit Olasz MD, PhD Associate Professor in the Dermatology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Animals
Animals, Newborn
Autoantibodies
Autoantigens
Autoimmune Diseases
Disease Models, Animal
Female
Humans
Maternal-Fetal Exchange
Mice
Non-Fibrillar Collagens
Pemphigoid, Bullous
Pregnancy