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@ARTICLE{Br:137318,
      author       = {Bär, Janina and Kumar, Vinod and Roth, Wera and Schwarz,
                      Nicole and Richter, Miriam and Leube, Rudolf E and Magin,
                      Thomas M},
      title        = {{S}kin fragility and impaired desmosomal adhesion in mice
                      lacking all keratins.},
      journal      = {The journal of investigative dermatology},
      volume       = {134},
      number       = {4},
      issn         = {0022-202X},
      address      = {Amsterdam},
      publisher    = {Elsevier},
      reportid     = {DZNE-2020-03640},
      pages        = {1012-1022},
      year         = {2014},
      abstract     = {Keratins perform major structural and regulatory functions
                      in epithelia. Owing to redundancy, their respective
                      contribution to epidermal integrity, adhesion, and cell
                      junction formation has not been addressed in full.
                      Unexpectedly, the constitutive deletion of type II keratins
                      in mice was embryonic lethal ∼ E9.5 without extensive
                      tissue damage. This prompted us to analyze keratin functions
                      in skin where keratins are best characterized. Here, we
                      compare the mosaic and complete deletion of all type II
                      keratins in mouse skin, with distinct consequences on
                      epidermal integrity, adhesion, and organismal survival.
                      Mosaic knockout (KO) mice survived ∼ 12 days while global
                      KO mice died perinatally because of extensive epidermal
                      damage. Coinciding with absence of keratins, epidermal
                      fragility, inflammation, increased epidermal thickness, and
                      increased proliferation were noted in both strains of mice,
                      accompanied by significantly smaller desmosomes. Decreased
                      desmosome size was due to accumulation of desmosomal
                      proteins in the cytoplasm, causing intercellular adhesion
                      defects resulting in intercellular splits. Mixing different
                      ratios of wild-type and KO keratinocytes revealed that ∼
                      $60\%$ of keratin-expressing cells were sufficient to
                      maintain epithelial sheets under stress. Our data reveal a
                      major contribution of keratins to the maintenance of
                      desmosomal adhesion and epidermal integrity with relevance
                      for the treatment of epidermolysis bullosa simplex and other
                      keratinopathies.},
      keywords     = {Animals / Cell Adhesion / Cell Membrane: metabolism /
                      Cytoplasm: metabolism / Desmosomes: metabolism / Disease
                      Models, Animal / Epidermis: metabolism / Epidermolysis
                      Bullosa Simplex: metabolism / Epithelium: metabolism / Gene
                      Deletion / Gene Expression Regulation, Developmental /
                      Keratinocytes: metabolism / Keratins, Type II: genetics /
                      Keratins, Type II: metabolism / Mice / Mice, Knockout /
                      Mosaicism / Phenotype / Skin: embryology / Skin: pathology /
                      Keratins, Type II (NLM Chemicals)},
      cin          = {AG Fava 1},
      ddc          = {610},
      cid          = {I:(DE-2719)1013016},
      pnm          = {342 - Disease Mechanisms and Model Systems (POF3-342)},
      pid          = {G:(DE-HGF)POF3-342},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:24121403},
      doi          = {10.1038/jid.2013.416},
      url          = {https://pub.dzne.de/record/137318},
}