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@ARTICLE{Huang:300226,
      author       = {Z. Huang and C. He and G. Wang and M. Zhu and X. Tong and
                      Y. Feng and C. Zhang and S. Dong and Y. Harim$^*$ and H.
                      Liu$^*$ and W. Zhou and F. Lan and W. Feng},
      title        = {{M}utation of {CHD}7 impairs the output of neuroepithelium
                      transition that is reversed by the inhibition of {EZH}2.},
      journal      = {Molecular psychiatry},
      volume       = {30},
      number       = {9},
      issn         = {1359-4184},
      address      = {[London]},
      publisher    = {Springer Nature},
      reportid     = {DKFZ-2025-00688},
      pages        = {4094-4109},
      year         = {2025},
      note         = {DKFZ-ZMBH Alliance /2025 Sep;30(9):4094-4109},
      abstract     = {Haploinsufficiency of CHD7 (Chromo-Helicase-DNA binding
                      protein 7) causes a severe congenital disease CHARGE
                      syndrome. Brain anomaly such as microcephaly and olfactory
                      bulb agenesis seen in CHARGE patients have not been mimicked
                      in previous animal models. Here, we uncover an indispensable
                      function of CHD7 in the neuroepithelium (NE) but not in the
                      neural stem cells (NSCs) after NE transition. Loss of Chd7
                      in mouse NE resulted in CHARGE-like brain anomalies due to
                      reduced proliferation and differentiation of neural stem and
                      progenitor cells, which were recapitulated in CHD7 KO human
                      forebrain organoids. Mechanistically, we find that CHD7
                      activates neural transcription factors by removing the
                      repressive histone mark H3K27me3 and promoting chromatin
                      accessibility. Importantly, neurodevelopmental defects
                      caused by CHD7 loss in human brain organoids and mice were
                      ameliorated by the inhibition of H3K27me3 methyltransferase
                      EZH2. Altogether, by implementing appropriate experimental
                      models, we uncover the pathogenesis of CHD7-associated
                      neurodevelopmental diseases, and identify a potential
                      therapeutic opportunity for CHARGE syndrome.},
      cin          = {A240},
      ddc          = {610},
      cid          = {I:(DE-He78)A240-20160331},
      pnm          = {311 - Zellbiologie und Tumorbiologie (POF4-311)},
      pid          = {G:(DE-HGF)POF4-311},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:40164694},
      doi          = {10.1038/s41380-025-02990-6},
      url          = {https://inrepo02.dkfz.de/record/300226},
}