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Fig 1.

Immunization with murine desmoglein 3 induces antibodies against Dsg3 but no clinical PV phenotype.

(A) C57Bl/6J, B6.SJL-H2s, DBA2/J or SJL/J mice were immunized 4x with mDsg3 emulsified in TiterMax on days 0, 14, 35, 56. (B) Dsg3-specific IgG autoantibody titers of C57Bl/6J (n = 4), B6.SJL-H2s (n = 4), DBA2/J (n = 4) and SJL/J (n = 4) mice immunized 4x with mDsg3 emulsified in TiterMax on days 0, 14, 35, and 56. (C) Direct immunofluorescent staining for anti-mouse-IgG (facial skin) at day 84. (D) Monkey esophagus was incubated with serum from immunized mice (day 84) and stained for anti-mouse-IgG. In both direct and indirect IF microscopy, no binding of IgG to the epidermis was detected. Dotted line: Dermal-epidermal junction. (E) Representative picture of endoscopy of the oral cavity showed no mucous membrane lesions at day 84. (F) The formation of mDsg3-specific B or plasma cells was confirmed by immunofluorescence of draining lymph nodes (day 84). B220: red, recombinant mDsg3 protein: green, arrows indicate Dsg3-specific B cells.

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Fig 2.

Immunization with murine desmoglein 3 and additional ETA or human desmoglein induce antibodies against Dsg3, but no clinical PV phenotype.

(A) Modified 4x immunization protocol (additional injection of 0.5 μg/ml ETA at day 28) was tested in DBA2/J and SJL/J mice on day 0, 14, 35, 56. The s.c. immunization was carried out with 100 μg mDsg3 emulsified in TiterMax. (B) Dsg3-specific IgG autoantibody titers of DBA2/J mice (n = 4) and SJL/J mice (n = 3). (C) Modified 4x immunization protocol was tested in DBA2/J and SJL/J mice on day 0, 14, 35, 56. The s.c. immunization was carried out with 30 μg human (h) Dsg1 and 70 μg hDsg3 emulsified in TiterMax (C). IgG autoantibody titers of DBA2/J mice (n = 4) and SJL/J (n = 5) were analyzed for their activity against (D) hDsg1, (E) hDsg3 and (F) cross-reactivity with mDsg3.

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Fig 2 Expand