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@ARTICLE{Hudemann:169841,
      author       = {C. Hudemann and R. Maglie and M. Llamazares$^*$ and B.
                      Beckert and D. Didona and R. Tikkanen and T. Schmitt and T.
                      Hashimoto and J. Waschke and M. Hertl and R. Eming},
      title        = {{H}uman desmocollin 3-specific {I}g{G} antibodies are
                      pathogenic in a humanized {HLA}-class {II} transgenic mouse
                      model of pemphigus.},
      journal      = {The journal of investigative dermatology},
      volume       = {142},
      number       = {3, Part B},
      issn         = {0022-202X},
      address      = {Amsterdam},
      publisher    = {Elsevier},
      reportid     = {DKFZ-2021-01584},
      pages        = {915-923.e3},
      year         = {2022},
      note         = {Volume 142, Issue 3, Part B, March 2022, Pages 915-923.e3},
      abstract     = {Pemphigus is a potentially lethal autoimmune bullous skin
                      disorder, which is associated with IgG autoantibodies
                      against desmoglein 3 (Dsg3) and Dsg1. Notably, a subset of
                      pemphigus patients presents with a similar clinical
                      phenotype in the absence of anti-Dsg IgG, suggesting the
                      presence of serum IgG reactive with desmosomal components
                      other than Dsg1 or Dsg3. We and others have previously shown
                      that such patients have serum IgG autoantibodies against
                      desmocollin 3 (Dsc3), a component of desmosomes, that induce
                      loss of keratinocyte adhesion ex vivo. Moreover, Dsc3
                      hypomorphic mice show a severe blistering phenotype of the
                      mucous membrane which is highly characteristic in pemphigus.
                      These findings prompted us to study induction and regulation
                      of anti-human Dsc3 IgG in humanized mice transgenic for
                      HLA-DRB1*04:02, which is a highly prevalent haplotype in
                      pemphigus. We show that IgG from sera of immunized mice
                      induce acantholysis in a dispase-based keratinocyte
                      dissociation assay via the activation of p38
                      mitogen-activated protein kinases and epidermal growth
                      factor receptor. Passive IgG transfer from mice immunized
                      with recombinant human Dsc3 into neonates did not induce
                      intraepidermal loss of adhesion presumably due to the lack
                      of homology between human and mouse Dsc3. Ex vivo
                      stimulation of splenocytes from Dsc3-immunized mice with
                      human Dsc3 leads to a significant proliferative
                      interferon-γ and interleukin 4 T cell response, which is
                      restricted by HLA-DR/DQ. These findings suggest that
                      induction of pathogenic anti-Dsc3 IgG is associated with
                      Dsc3-specific T cells that recognize Dsc3 in association
                      with HLA-DRB1*04:02.},
      keywords     = {HLA (Other) / antibodies (Other) / autoimmunity (Other) /
                      desmocollin (Other) / pemphigus vulgaris (Other)},
      cin          = {B370},
      ddc          = {610},
      cid          = {I:(DE-He78)B370-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:34265330},
      doi          = {10.1016/j.jid.2021.06.017},
      url          = {https://inrepo02.dkfz.de/record/169841},
}