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The transcription factor Sox10 is a key regulator of peripheral glial development

Official URL:http://genesdev.cshlp.org/content/15/1/66.abstract
PubMed:View item in PubMed
Creators Name:Britsch, S. and Goerich, D.E. and Riethmacher, D. and Peirano, R.I. and Rossner, M. and Nave, K.A. and Birchmeier, C. and Wegner, M.
Journal Title:Genes & Development
Journal Abbreviation:Genes Dev
Volume:15
Number:1
Page Range:66-78
Date:1 January 2001
Keywords:Cell Differentiation, Chimera, DNA-Binding Proteins, Spinal Ganglia, Developmental Gene Expression Regulation, Heterozygote, High Mobility Group Proteins, Homozygote, Neural Crest, Neuroblastoma, Neuroglia, erbB-3 Receptor, SOXE Transcription Factors, Transcription Factors, Cultured Tumor Cells, beta-Galactosidase, Animals, Mice, Rats
Abstract:The molecular mechanisms that determine glial cell fate in the vertebrate nervous system have not been elucidated. Peripheral glial cells differentiate from pluripotent neural crest cells. We show here that the transcription factor Sox10 is a key regulator in differentiation of peripheral glial cells. In mice that carry a spontaneous or a targeted mutation of Sox10, neuronal cells form in dorsal root ganglia, but Schwann cells or satellite cells are not generated. At later developmental stages, this lack of peripheral glial cells results in a severe degeneration of sensory and motor neurons. Moreover, we show that Sox10 controls expression of ErbB3 in neural crest cells. ErbB3 encodes a Neuregulin receptor, and down-regulation of ErbB3 accounts for many changes in development of neural crest cells observed in Sox10 mutant mice. Sox10 also has functions not mediated by ErbB3, for instance in the melanocyte lineage. Phenotypes observed in heterozygous mice that carry a targeted Sox10 null allele reproduce those observed in heterozygous Sox10(Dom) mice. Haploinsufficiency of Sox10 can thus cause pigmentation and megacolon defects, which are also observed in Sox10(Dom)/+ mice and in patients with Waardenburg-Hirschsprung disease caused by heterozygous SOX10 mutations.
ISSN:0890-9369
Publisher:Cold Spring Harbor Laboratory Press (U.S.A.)
Item Type:Article

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