Abstract
Bullous pemphigoid (BP) and linear IgA disease (LAD) are autoimmune subepidermal blistering skin disease associated with autoantibodies against the transmembrane hemidesmosomal protein BP180/type XVII collagen. It has been demonstrated previously that BP is characterized predominantly by IgG autoantibodies, while autoantibodies in LAD mainly belong to the IgA isotype. The aim of the present study was to investigate the hypothesis that there is a significant overlap in the autoantibody isotype associated with these two disease. Several new recombinant forms of BP180 were generated in the baculovirus expression system, including the full-length protein. IgG autoantibodies to BP 180 were detectable in 39 of 40 (98%) of BP sera; interestingly, 88% of BP sera also contained IgA anti-BP180 autoantibodies. Similarly, anit-BP180 reactivity in LAD sera (n=22) was also attributed to both an IgA (68%) and an IgG (76%) autoanitbody response. IgA and IgG autoantibodies to the intracellular portion of BP180 were found in 14% and 28% of BP sera, respectively, and in 8% of LAD sera (same percentage for both isotypes). Our findings clearly demonstrate that both BP and LAD patients have dual IgA and IgG autoimmune response to BP180 which is directed not only to the ectodomain, but also to the intracellular portion of this protein. (C) 2000 Academic Press.
| Original language | English |
|---|---|
| Journal | Journal of Autoimmunity |
| Volume | 15 |
| Issue number | 3 |
| Pages (from-to) | 293-300 |
| Number of pages | 8 |
| ISSN | 0896-8411 |
| DOIs | |
| Publication status | Published - 2000 |
Funding
This work has been supported by grant 98.073.1 from the Wilhelm Sander-Stiftung, Munich, Germany (D.Z.). We thank Drs Gerald Messer and Karin Partscht, Munich, Germany, for providing us with serum samples from patients with linear IgA disease and Drs George J. Giudice and Luis A. Diaz, Milwaukee, WI, USA, for providing us with rabbit sera 136 and 594. We are also grateful to Christa Knaus, Würzburg, for assistance producing LAD-1 from conditioned HaCaT cell medium, and Hartmut Wulf, Hamburg, for help preparing the figures.
UN SDGs
This output contributes to the following UN Sustainable Development Goals (SDGs)
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SDG 3 Good Health and Well-being
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