Sox10 promotes the survival of cochlear progenitors during the establishment of the organ of Corti. Dev Biol

GIGA-Neurosciences, University of Liege, Belgium.
Developmental Biology (Impact Factor: 3.64). 09/2009; 335(2):327-39. DOI: 10.1016/j.ydbio.2009.09.007
Source: PubMed

ABSTRACT Transcription factors of the SoxE family are critical players that underlie various embryological processes. However, little is known about their function during inner ear development. Here, we show that Sox10 is initially expressed throughout the otic vesicle epithelium and becomes later restricted to supporting cells as cell differentiation proceeds in the organ of Corti. Morphological analyses of Sox10 mutant mice reveal a significant shortening of the cochlear duct likely resulting from the progressive depletion of cochlear progenitors. While Sox10 appears dispensable for the differentiation and patterning of the inner ear prosensory progenitors, our data support a critical role for this transcription factor in the promotion of their survival. We provide genetic evidences that Sox10, in a concentration-dependant manner, could play a role in the regulation of Jagged1, a gene known to be important for inner ear prosensory development. Together, our results demonstrate that Sox10 regulates the biology of early cochlear progenitors during inner ear development, but, in contrast to neural crest-derived cells, this transcription factor is dispensable for their differentiation. Evidence also suggests that this effect occurs via the activation of the Jagged1 gene.

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Available from: Laurent Nguyen, Aug 27, 2015
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    • "However, early and wide SOX10 expression during the inner ear development [Breuskin et al., 2009; Dutton et al., 2009; Watanabe et al., 2000] suggests that other mechanisms may account for deafness associated with SOX10 mutations. A shortening of the cochlea due to a reduced sensory progenitor survival has recently been described in the Sox10 knock-out mouse [Breuskin et al., 2009]. These results are consistent with the observation that SOX10 mutations in human lead to morphological abnormalities of the inner ear at the MRI or CT-scan ([Barnett et al., 2009; Inoue et al., 2004; Pingault et al., 2002; Sznajer et al., 2008; Vinuela et al., 2009] and manuscript in preparation). "
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