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@ARTICLE{Jenseit:177381,
      author       = {A. L. Jenseit$^*$ and A. Camgoz$^*$ and S. M. Pfister$^*$
                      and M. Kool$^*$},
      title        = {{EZHIP}: a new piece of the puzzle towards understanding
                      pediatric posterior fossa ependymoma.},
      journal      = {Acta neuropathologica},
      volume       = {143},
      number       = {1},
      issn         = {1432-0533},
      address      = {Heidelberg},
      publisher    = {Springer},
      reportid     = {DKFZ-2021-02473},
      pages        = {1-13},
      year         = {2022},
      note         = {#EA:B062#LA:B062# / 2022 Jan;143(1):1-13},
      abstract     = {Ependymomas (EPN) are tumors of the central nervous system
                      (CNS) that can arise in the supratentorial brain (ST-EPN),
                      hindbrain or posterior fossa (PF-EPN) or anywhere in the
                      spinal cord (SP-EPN), both in children and adults.
                      Molecular profiling studies have identified distinct groups
                      and subtypes in each of these anatomical compartments. In
                      this review, we give an overview on recent findings and new
                      insights what is driving PFA ependymomas, which is the most
                      common group. PFA ependymomas are characterized by a young
                      median age at diagnosis, an overall balanced genome and a
                      bad clinical outcome $(56\%$ 10-year overall survival).
                      Sequencing studies revealed no fusion genes or other highly
                      recurrently mutated genes, suggesting that the disease is
                      epigenetically driven. Indeed, recent findings have shown
                      that the characteristic global loss of the repressive
                      histone 3 lysine 27 trimethylation (H3K27me3) mark in PFA
                      ependymoma is caused by aberrant expression of the enhancer
                      of zeste homolog inhibitory protein (EZHIP) or in rare cases
                      by H3K27M mutations, which both inhibit EZH2 thereby
                      preventing the polycomb repressive complex 2 (PRC2) from
                      spreading H3K27me3. We present the current status of the
                      ongoing work on EZHIP and its essential role in the
                      epigenetic disturbance of PFA biology. Comparisons to the
                      oncohistone H3K27M and its role in diffuse midline glioma
                      (DMG) are drawn, highlighting similarities but also
                      differences between the tumor entities and underlying
                      mechanisms. A strong focus is to point out missing
                      information and to present directions of further research
                      that may result in new and improved therapies for PFA
                      ependymoma patients.},
      subtyp        = {Review Article},
      keywords     = {DMG (Other) / EZHIP (Other) / H3K27M (Other) / H3K27me3
                      (Other) / PFA ependymoma (Other) / PRC2 (Other)},
      cin          = {B062},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:34762160},
      doi          = {10.1007/s00401-021-02382-4},
      url          = {https://inrepo02.dkfz.de/record/177381},
}