title: An attempt to develop mouse model for anti-laminin γ1 pemphigoid.
abstract: BACKGROUND
  We recently reported that the autoantibodies of anti-p200 pemphigoid sera react with laminin γ1 and renamed this entity as anti-laminin γ1 pemphigoid. However, it has not been clarified whether the anti-laminin γ1 autoantibodies, particularly those to the C-terminal integrin binding site, affect the dermoepidermal junction and cause subepidermal blisters.
  
  
  OBJECTIVE
  The aim of this study was to develop animal models for anti-laminin γ1 pemphigoid.
  
  
  METHODS
  We attempted to produce two mouse models for anti-laminin γ1 pemphigoid; (1) a passive transfer model: injection of rabbit IgG to shorter bacterial recombinant protein of the murine laminin γ1 C-terminal 107 amino acids, and (2) an active disease model: direct immunization to mice with this recombinant protein.
  
  
  RESULTS
  Immunoblotting revealed that 70% of patient sera reacted with the shorter recombinant protein of human laminin γ1 C-terminus. In the passive transfer model, rabbit IgG to the murine laminin γ1 C-terminus was deposited, without C3 deposition, at the epidermal basement membrane zone. In contrast, in the active disease model, direct immunofluorescence of mouse skin sections showed no deposition of either murine IgG or C3. Blister formation was not seen in either model both phenotypically and histopathologically.
  
  
  CONCLUSIONS
  In the two different mouse animal models for anti-laminin γ1 pemphigoid, although rabbit IgG to the recombinant laminin γ1 C-terminus bound to the epidermal basement membrane zone in passive transfer model, no obvious blister formation was seen. To reproduce skin lesions in mouse models for anti-laminin γ1 pemphigoid, further improvement should be needed.
authors: Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.
authors: Hiroshi
authors: H
authors: Koga
authors: Norito
authors: N
authors: Ishii
authors: Teruki
authors: T
authors: Dainichi
authors: Daisuke
authors: D
authors: Tsuruta
authors: Takahiro
authors: T
authors: Hamada
authors: Chika
authors: C
authors: Ohata
authors: Tadashi
authors: T
authors: Karashima
authors: Minao
authors: M
authors: Furumura
authors: Takashi
authors: T
authors: Hashimoto
mesh_terms: D000596:Amino Acids / Q000276:immunology; D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D004195:Disease Models, Animal; D006801:Humans; D007110:Immunity, Active / Q000276:immunology; D007116:Immunization, Passive; D007074:Immunoglobulin G / Q000276:immunology; D007797:Laminin / Q000276:immunology*; D051379:Mice; D008807:Mice, Inbred BALB C; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D010391:Pemphigoid, Bullous / Q000276:immunology*; D011817:Rabbits; D011994:Recombinant Proteins / Q000276:immunology
chemical_list: D000596:Amino Acids; D001323:Autoantibodies; D007074:Immunoglobulin G; D007797:Laminin; D011994:Recombinant Proteins; C089141:laminin gamma 1