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The humanized SCID mouse model to study HLA class II‐linked autoimmunity to desmoglein 3 in pemphigus vulgaris
Author(s) -
RÄdisch T.,
Riechers R.,
Hertl M.
Publication year - 2002
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.2002.04568.x
Subject(s) - pemphigus vulgaris , desmoglein 3 , antibody , immunology , medicine , pemphigus , autoimmunity , in vivo , desmoglein , autoantibody , biology , microbiology and biotechnology
Summary Background Pemphigus vulgaris (PV) is a severe autoimmmune skin disorder induced by antibodies against desmoglein (Dsg) 3 on epidermal keratinocytes. Objectives To establish an active animal model of PV to analyse the T‐cell‐regulated production of pathogenic antibodies in vivo . Methods Immunodeficient SCID mice were injected with peripheral blood lymphocytes (PBL) from an HLA‐DRB1*0402/DQ8+ patient with PV or a DRB1*0402/DQ8+ healthy donor, with or without subsequent injections of human Dsg3 or preincubation of PBL with Dsg3. Results Human immunoglobulins (2·7–18·5 mg mL −1 ) were detected in all the mice after 8 weeks. Only one of 30 PBL‐treated mice developed IgM against Dsg3 and showed intercellular IgM deposits in skin, nostrils and tongue. In contrast, in a previous study, 41% of SCID mice injected with PBL from patients with PV developed anti‐Dsg3 antibodies in vivo . Conclusions Our inability to reproduce these findings may be due to the transfer of slightly lower numbers of PBL (20 × 10 6 vs. 25–30 × 10 6 ).