@ARTICLE{Bershteyn2017-ib,
  title    = "Human {iPSC-Derived} Cerebral Organoids Model Cellular Features
              of Lissencephaly and Reveal Prolonged Mitosis of Outer Radial
              Glia",
  author   = "Bershteyn, Marina and Nowakowski, Tomasz J and Pollen, Alex A and
              Di Lullo, Elizabeth and Nene, Aishwarya and Wynshaw-Boris,
              Anthony and Kriegstein, Arnold R",
  abstract = "Classical lissencephaly is a genetic neurological disorder
              associated with mental retardation and intractable epilepsy, and
              Miller-Dieker syndrome (MDS) is the most severe form of the
              disease. In this study, to investigate the effects of MDS on
              human progenitor subtypes that control neuronal output and
              influence brain topology, we analyzed cerebral organoids derived
              from control and MDS-induced pluripotent stem cells (iPSCs) using
              time-lapse imaging, immunostaining, and single-cell RNA
              sequencing. We saw a cell migration defect that was rescued when
              we corrected the MDS causative chromosomal deletion and severe
              apoptosis of the founder neuroepithelial stem cells, accompanied
              by increased horizontal cell divisions. We also identified a
              mitotic defect in outer radial glia, a progenitor subtype that is
              largely absent from lissencephalic rodents but critical for human
              neocortical expansion. Our study, therefore, deepens our
              understanding of MDS cellular pathogenesis and highlights the
              broad utility of cerebral organoids for modeling human
              neurodevelopmental disorders.",
  journal  = "Cell Stem Cell",
  volume   =  20,
  number   =  4,
  pages    = "435--449.e4",
  month    =  jan,
  year     =  2017,
  keywords = "cerebral organoids; human lissencephaly; migration; outer radial
              glia; spindle orientation",
  language = "en"
}

{"_id":"WPHmEjCbPdDBf7fAk","bibbaseid":"bershteyn-nowakowski-pollen-dilullo-nene-wynshawboris-kriegstein-humanipscderivedcerebralorganoidsmodelcellularfeaturesoflissencephalyandrevealprolongedmitosisofouterradialglia-2017","authorIDs":[],"author_short":["Bershteyn, M.","Nowakowski, T. J","Pollen, A. A","Di Lullo, E.","Nene, A.","Wynshaw-Boris, A.","Kriegstein, A. R"],"bibdata":{"bibtype":"article","type":"article","title":"Human iPSC-Derived Cerebral Organoids Model Cellular Features of Lissencephaly and Reveal Prolonged Mitosis of Outer Radial Glia","author":[{"propositions":[],"lastnames":["Bershteyn"],"firstnames":["Marina"],"suffixes":[]},{"propositions":[],"lastnames":["Nowakowski"],"firstnames":["Tomasz","J"],"suffixes":[]},{"propositions":[],"lastnames":["Pollen"],"firstnames":["Alex","A"],"suffixes":[]},{"propositions":[],"lastnames":["Di","Lullo"],"firstnames":["Elizabeth"],"suffixes":[]},{"propositions":[],"lastnames":["Nene"],"firstnames":["Aishwarya"],"suffixes":[]},{"propositions":[],"lastnames":["Wynshaw-Boris"],"firstnames":["Anthony"],"suffixes":[]},{"propositions":[],"lastnames":["Kriegstein"],"firstnames":["Arnold","R"],"suffixes":[]}],"abstract":"Classical lissencephaly is a genetic neurological disorder associated with mental retardation and intractable epilepsy, and Miller-Dieker syndrome (MDS) is the most severe form of the disease. In this study, to investigate the effects of MDS on human progenitor subtypes that control neuronal output and influence brain topology, we analyzed cerebral organoids derived from control and MDS-induced pluripotent stem cells (iPSCs) using time-lapse imaging, immunostaining, and single-cell RNA sequencing. We saw a cell migration defect that was rescued when we corrected the MDS causative chromosomal deletion and severe apoptosis of the founder neuroepithelial stem cells, accompanied by increased horizontal cell divisions. We also identified a mitotic defect in outer radial glia, a progenitor subtype that is largely absent from lissencephalic rodents but critical for human neocortical expansion. Our study, therefore, deepens our understanding of MDS cellular pathogenesis and highlights the broad utility of cerebral organoids for modeling human neurodevelopmental disorders.","journal":"Cell Stem Cell","volume":"20","number":"4","pages":"435–449.e4","month":"January","year":"2017","keywords":"cerebral organoids; human lissencephaly; migration; outer radial glia; spindle orientation","language":"en","bibtex":"@ARTICLE{Bershteyn2017-ib,\n title = \"Human {iPSC-Derived} Cerebral Organoids Model Cellular Features\n of Lissencephaly and Reveal Prolonged Mitosis of Outer Radial\n Glia\",\n author = \"Bershteyn, Marina and Nowakowski, Tomasz J and Pollen, Alex A and\n Di Lullo, Elizabeth and Nene, Aishwarya and Wynshaw-Boris,\n Anthony and Kriegstein, Arnold R\",\n abstract = \"Classical lissencephaly is a genetic neurological disorder\n associated with mental retardation and intractable epilepsy, and\n Miller-Dieker syndrome (MDS) is the most severe form of the\n disease. In this study, to investigate the effects of MDS on\n human progenitor subtypes that control neuronal output and\n influence brain topology, we analyzed cerebral organoids derived\n from control and MDS-induced pluripotent stem cells (iPSCs) using\n time-lapse imaging, immunostaining, and single-cell RNA\n sequencing. We saw a cell migration defect that was rescued when\n we corrected the MDS causative chromosomal deletion and severe\n apoptosis of the founder neuroepithelial stem cells, accompanied\n by increased horizontal cell divisions. We also identified a\n mitotic defect in outer radial glia, a progenitor subtype that is\n largely absent from lissencephalic rodents but critical for human\n neocortical expansion. Our study, therefore, deepens our\n understanding of MDS cellular pathogenesis and highlights the\n broad utility of cerebral organoids for modeling human\n neurodevelopmental disorders.\",\n journal = \"Cell Stem Cell\",\n volume = 20,\n number = 4,\n pages = \"435--449.e4\",\n month = jan,\n year = 2017,\n keywords = \"cerebral organoids; human lissencephaly; migration; outer radial\n glia; spindle orientation\",\n language = \"en\"\n}\n\n","author_short":["Bershteyn, M.","Nowakowski, T. J","Pollen, A. A","Di Lullo, E.","Nene, A.","Wynshaw-Boris, A.","Kriegstein, A. R"],"key":"Bershteyn2017-ib","id":"Bershteyn2017-ib","bibbaseid":"bershteyn-nowakowski-pollen-dilullo-nene-wynshawboris-kriegstein-humanipscderivedcerebralorganoidsmodelcellularfeaturesoflissencephalyandrevealprolongedmitosisofouterradialglia-2017","role":"author","urls":{},"keyword":["cerebral organoids; human lissencephaly; migration; outer radial glia; spindle orientation"],"metadata":{"authorlinks":{}},"downloads":0},"bibtype":"article","biburl":"https://bibbase.org/f/EJMp3HRuxirjxpcXh/references.bib","creationDate":"2020-07-02T10:44:34.888Z","downloads":0,"keywords":["cerebral organoids; human lissencephaly; migration; outer radial glia; spindle orientation"],"search_terms":["human","ipsc","derived","cerebral","organoids","model","cellular","features","lissencephaly","reveal","prolonged","mitosis","outer","radial","glia","bershteyn","nowakowski","pollen","di lullo","nene","wynshaw-boris","kriegstein"],"title":"Human iPSC-Derived Cerebral Organoids Model Cellular Features of Lissencephaly and Reveal Prolonged Mitosis of Outer Radial Glia","year":2017,"dataSources":["QogkwkhcTZyMJJoTw","sAFYeB74DpbdXM9NN","4zx9n2tbeLTix3Wxr","k3cdWrThyTh5o59Rm","hq9pebjzmsTuyxGGx","h8Atv2SAy4PmShg5j"]}