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Equine bullous pemphigoid IgG autoantibodies target linear epitopes in the NC16A ectodomain of collagen XVII (BP180, BPAG2).

Abstract
Bullous pemphigoid (BP) is an autoimmune subepithelial blistering dermatosis of humans, dogs, cats and pigs. It is characterized by skin-fixed and circulating IgG autoantibodies that target one or both BP antigens. An immunological homologue of BP in humans was diagnosed in two horses with cutaneous and mucosal ulcerations as well as microscopic subepithelial vesiculation. Immunological investigations revealed similar findings for both the horses. Direct immunofluorescence demonstrated the presence of IgG deposited linearly at the dermoepidermal junction in mucosal and skin biopsy specimens. Indirect immunofluorescence testing confirmed the existence of circulating basement membrane-specific IgG autoantibodies. Using intact and salt-split epithelial substrates, serum IgG were shown to target antigens situated not only at the basal, but also at the lateral and apical aspects of stratum basale keratinocytes. Immunoblotting and ELISA corroborated that the IgG from affected horses, but not those from normal controls, exhibited high immunoreactivity against the NC16A extracellular domain of type XVII collagen (BPAG2, BP180). Equine BP could be proposed, therefore, as another spontaneous model of this most common basement membrane autoimmune dermatosis of humans.
AuthorsT Olivry, A K Borrillo, L Xu, S M Dunston, N M Slovis, V K Affolter, T C Demanuelle, L S Chan
JournalVeterinary immunology and immunopathology (Vet Immunol Immunopathol) Vol. 73 Issue 1 Pg. 45-52 (Jan 31 2000) ISSN: 0165-2427 [Print] Netherlands
PMID10678397 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Autoantibodies
  • Autoantigens
  • Carrier Proteins
  • Cytoskeletal Proteins
  • Dst protein, mouse
  • Dystonin
  • Epitopes
  • Immunoglobulin G
  • Nerve Tissue Proteins
  • Non-Fibrillar Collagens
  • Collagen
Topics
  • Animals
  • Autoantibodies (analysis, immunology)
  • Autoantigens (immunology)
  • Carrier Proteins
  • Collagen
  • Cytoskeletal Proteins
  • Dystonin
  • Enzyme-Linked Immunosorbent Assay
  • Epitopes
  • Horse Diseases (immunology)
  • Horses
  • Immunoblotting
  • Immunoglobulin G (analysis, immunology)
  • Microscopy, Fluorescence
  • Nerve Tissue Proteins
  • Non-Fibrillar Collagens
  • Pemphigoid, Bullous (immunology, veterinary)
  • Collagen Type XVII

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