Genes & Development · 2002 · 682 citations · 26 references
Life SciencesGeneticsNeuroscienceDevelopmental NeuroscienceGlial BiologyMolecular NeuroscienceGlial Cell BiologyGlial DevelopmentNervous TissueNeural DevelopmentNeural Stem CellsOligodendrocyte BiologyMyelin Gene ExpressionMyelin Gene RegulationMyelin-associated Gene ExpressionOligodendrocyte DevelopmentOligodendrocyte Gene ExpressionOligodendrocyte Gene RegulationOligodendrocyte Progenitor CellsPeripheral Nervous System Myelination
Sox10 is a high‑mobility‑group transcriptional regulator expressed in early neural crest. Sox10 is required for glial development: its loss prevents peripheral glia formation, limits CNS expression to myelin‑forming oligodendroglia, disrupts terminal differentiation of progenitors, abolishes myelin production even when Sox10‑deficient neural stem cells are transplanted into wild‑type hosts, directly activates myelin genes in oligodendrocytes, but does not regulate erbB3, revealing distinct roles in peripheral and central glia.
Sox10 is a high-mobility-group transcriptional regulator in early neural crest. Without Sox10, no glia develop throughout the peripheral nervous system. Here we show that Sox10 is restricted in the central nervous system to myelin-forming oligodendroglia. In Sox10-deficient mice progenitors develop, but terminal differentiation is disrupted. No myelin was generated upon transplantation of Sox10-deficient neural stem cells into wild-type hosts showing the permanent, cell-autonomous nature of the defect. Sox10 directly regulates myelin gene expression in oligodendrocytes, but does not control erbB3 expression as in peripheral glia. Sox10 thus functions in peripheral and central glia at different stages and through different mechanisms.
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The transcription factor Sox10 is a key regulator of peripheral glial development
Stefan Britsch, Derk E. Goerich, Dieter Riethmacher et al. · Genes & Development · 2001 · 908 citations · Full text
Medicine, Genetics, Neuroscience +14
SOX10 mutations in patients with Waardenburg-Hirschsprung disease
Véronique Pingault, Nadège Bondurand, Kirsten Kuhlbrodt et al. · Nature Genetics · 1998 · 818 citations
Sox10, a Novel Transcriptional Modulator in Glial Cells
Kirsten Kuhlbrodt, Beate Herbarth, Elisabeth Sock et al. · Journal of Neuroscience · 1998 · 788 citations · Full text