Glial but not neuronal development in the cochleo-vestibular ganglion requires Sox10.

The cochleo-vestibular ganglion contains neural crest-derived glial cells and sensory neurons that are derived from the neurogenic otic placode. Little is known about the molecular mechanisms that regulate the tightly orchestrated development of this structure. Here, we report that Sox10, a high-mobility group DNA-binding domain transcription factor that is required for the proper development of neural crest cell derivatives, is specifically expressed in post-migratory neural crest cells in the cochleo-vestibular ganglion.

A short upstream promoter region mediates transcriptional regulation of the mouse doublecortin gene in differentiating neurons.

Background: Doublecortin (Dcx), a MAP (Microtubule-Associated Protein), is transiently expressed in migrating and differentiating neurons and thereby characterizes neuronal precursors and neurogenesis in developing and adult neurogenesis. In addition, reduced Dcx expression during development has been related to appearance of brain pathologies. Here, we attempt to unveil the molecular mechanisms controlling Dcx gene expression by studying its transcriptional regulation during neuronal differentiation.

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

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.

Expression patterns of miR-96, miR-182 and miR-183 in the development inner ear.

MicroRNAs (miRNAs) constitute a class of small non-coding endogenous RNAs that downregulate gene expression by binding to 3' untranslated region (UTR) of target messenger RNAs. Although they have been found to regulate developmental and physiological processes in several organs and tissues, their role in the regulation of the inner ear transcriptome remains unknown. In this report, we have performed systematic in situ hybridization to analyze the temporal and spatial distribution of three miRNAs (miR-96, mR-182, and mR-183) that are likely to arise from a single precursor RNA during the development and the maturation of the cochlea.

Hair cell progenitors: identification and regulatory genes.

Hair cell loss in higher vertebrates appears to be permanent. Progenitors that are quiescent in the organ of Corti are the best candidates for the restoration of the different cell types in the organ of Corti. However, little is known about the presence of these progenitors and their capacity to differentiate into hair cells.

Strategies to regenerate hair cells: identification of progenitors and critical genes.

Deafness commonly results from a lesion of the sensory cells and/or of the neurons of the auditory part of the inner ear. There are currently no treatments designed to halt or reverse the progression of hearing loss. A key goal in developing therapy for sensorineural deafness is the identification of strategies to replace lost hair cells.