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@ARTICLE{Kant:861126,
      author       = {Kant, Sebastian and Freytag, Benjamin and Herzog, Antonia
                      and Reich, Anna and Merkel, Rudolf and Hoffmann, Bernd and
                      Krusche, Claudia A. and Leube, Rudolf E.},
      title        = {{D}esmoglein 2 mutation provokes skeletal muscle actin
                      expression and accumulation at intercalated discs in murine
                      hearts},
      journal      = {Journal of cell science},
      volume       = {132},
      number       = {5},
      issn         = {1477-9137},
      address      = {Cambridge},
      publisher    = {Company of Biologists Limited},
      reportid     = {FZJ-2019-01683},
      pages        = {jcs199612 -},
      year         = {2019},
      abstract     = {Arrhythmogenic cardiomyopathy (AC) is an incurable
                      progressive disease that is linked to mutations in genes
                      coding for components of desmosomal adhesions that are
                      localized to the intercalated disc region, which
                      electromechanically couples adjacent cardiomyocytes. To
                      date, the underlying molecular dysfunctions are not well
                      characterized. In two murine AC models, we find an
                      upregulation of the skeletal muscle actin gene (Acta1),
                      which is known to be a compensatory reaction to compromised
                      heart function. Expression of this gene is elevated prior to
                      visible morphological alterations and clinical symptoms, and
                      persists throughout pathogenesis with an additional major
                      rise during the chronic disease stage. We provide evidence
                      that the increased Acta1 transcription is initiated through
                      nuclear activation of the serum response transcription
                      factor (SRF) by its transcriptional co-activator
                      megakaryoblastic leukemia 1 protein (MKL1, also known as
                      MRTFA). Our data further suggest that perturbed desmosomal
                      adhesion causes Acta1 overexpression during the early stages
                      of the disease, which is amplified by transforming growth
                      factor β (TGFβ) release from fibrotic lesions and
                      surrounding cardiomyocytes during later disease stages.
                      These observations highlight a hitherto unknown molecular AC
                      pathomechanism.},
      cin          = {ICS-7},
      ddc          = {570},
      cid          = {I:(DE-Juel1)ICS-7-20110106},
      pnm          = {552 - Engineering Cell Function (POF3-552)},
      pid          = {G:(DE-HGF)POF3-552},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:30659114},
      UT           = {WOS:000461414300006},
      doi          = {10.1242/jcs.199612},
      url          = {https://juser.fz-juelich.de/record/861126},
}