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Huntington Disease's Glial Progenitor Cells Hit the Pause Button in the Mouse Brain
1Center for Neurobehavioral Genetics, Jane and Terry Semel Institute for Neuroscience and Human Behavior at UCLA, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA; Department of Psychiatry and Biobehavioral Sciences, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Cell Stem Cell
|January 5, 2019
Abstract:
White matter abnormalities are prominent neuropathological features in Huntington's disease (HD); however, the cellular culprits are unclear. In this issue of Cell Stem Cell, Osipovitch et al. (2019) show that patient-derived glial progenitor cells fail to properly differentiate and myelinate in the mouse brain due to cell-intrinsic transcriptional defects.