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@ARTICLE{Feldner:125248,
      author       = {A. Feldner$^*$ and M. G. Adam$^*$ and F. Tetzlaff$^*$ and
                      I. Moll$^*$ and D. Komljenovic$^*$ and F. Sahm$^*$ and T.
                      Bäuerle$^*$ and H. Ishikawa and H. Schroten and T. Korff
                      and I. Hofmann$^*$ and H. Wolburg and A. von Deimling$^*$
                      and A. Fischer$^*$},
      title        = {{L}oss of {M}pdz impairs ependymal cell integrity leading
                      to perinatal-onset hydrocephalus in mice.},
      journal      = {EMBO molecular medicine},
      volume       = {9},
      number       = {7},
      issn         = {1757-4684},
      address      = {Weinheim},
      publisher    = {Wiley-VCH},
      reportid     = {DKFZ-2017-01400},
      pages        = {890 - 905},
      year         = {2017},
      abstract     = {Hydrocephalus is a common congenital anomaly. LCAM1 and
                      MPDZ (MUPP1) are the only known human gene loci associated
                      with non-syndromic hydrocephalus. To investigate functions
                      of the tight junction-associated protein Mpdz, we generated
                      mouse models. Global Mpdz gene deletion or conditional
                      inactivation in Nestin-positive cells led to formation of
                      supratentorial hydrocephalus in the early postnatal period.
                      Blood vessels, epithelial cells of the choroid plexus, and
                      cilia on ependymal cells, which line the ventricular system,
                      remained morphologically intact in Mpdz-deficient brains.
                      However, flow of cerebrospinal fluid through the cerebral
                      aqueduct was blocked from postnatal day 3 onward. Silencing
                      of Mpdz expression in cultured epithelial cells impaired
                      barrier integrity, and loss of Mpdz in astrocytes increased
                      RhoA activity. In Mpdz-deficient mice, ependymal cells had
                      morphologically normal tight junctions, but expression of
                      the interacting planar cell polarity protein Pals1 was
                      diminished and barrier integrity got progressively lost.
                      Ependymal denudation was accompanied by reactive
                      astrogliosis leading to aqueductal stenosis. This work
                      provides a relevant hydrocephalus mouse model and
                      demonstrates that Mpdz is essential to maintain integrity of
                      the ependyma.},
      cin          = {A270 / E020 / G380 / A190 / L101},
      ddc          = {610},
      cid          = {I:(DE-He78)A270-20160331 / I:(DE-He78)E020-20160331 /
                      I:(DE-He78)G380-20160331 / I:(DE-He78)A190-20160331 /
                      I:(DE-He78)L101-20160331},
      pnm          = {311 - Signalling pathways, cell and tumor biology
                      (POF3-311)},
      pid          = {G:(DE-HGF)POF3-311},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:28500065},
      pmc          = {pmc:PMC5494508},
      doi          = {10.15252/emmm.201606430},
      url          = {https://inrepo02.dkfz.de/record/125248},
}