Immunoadsorption against two distinct epitopes on human type XVII collagen abolishes dermal-epidermal separation induced in vitro by autoantibodies from pemphigoid gestationis patients

Eur J Immunol. 2006 Apr;36(4):1039-48. doi: 10.1002/eji.200535349.

Abstract

Pemphigoid gestationis (PG) is a subepidermal autoimmune blistering disease characterized by self-reactive T and B cells specific for the transmembrane hemidesmosomal protein type XVII collagen/BP180. Major T and B cell epitopes are located within the immunodominant 16th non-collagenous domain A (NC16A) of type XVII collagen. The aim of the present study was to map the pathogenically relevant epitopes targeted by blister-inducing patients' autoantibodies. For this purpose, we used an in vitro model of autoantibody-induced leukocyte-dependent dermal-epidermal separation. Pre-adsorption against a recombinant form of the NC16A region abolished the blister-inducing potential of autoantibodies from all PG patients. Using overlapping synthetic peptides, we demonstrated that PG autoantibodies bind to two defined epitopes within the NC16A region (aa 500-514 and aa 511-523). Importantly, pre-adsorption using an affinity matrix containing these epitopes completely abolished dermal-epidermal separation induced by PG autoantibodies. This study identifies the epitopes relevant for blister induction in PG and should facilitate the development of an antigen-specific immunoadsorption therapy for this disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibody Specificity
  • Autoantibodies / immunology*
  • Collagen Type XVIII / genetics
  • Collagen Type XVIII / immunology*
  • Dermis / immunology
  • Dermis / pathology*
  • Enzyme-Linked Immunosorbent Assay
  • Epidermis / immunology
  • Epidermis / pathology*
  • Epitopes / immunology*
  • Female
  • Humans
  • Immunoblotting
  • Immunosorbent Techniques
  • In Vitro Techniques
  • Pemphigoid Gestationis / immunology*
  • Pemphigoid Gestationis / pathology
  • Pregnancy

Substances

  • Autoantibodies
  • Collagen Type XVIII
  • Epitopes