Presentation
Sylvain Ernest is interested in genetic deafness. He has studied Myo7a, a gene implicated in Usher syndrome, a syndrome associating early onset deafness and blindness at puberty. He uses the zebrafish as animal model for deciphering the role of genes, whose malfunction is responsible for deafness.
Since his arrival at Imagine Institute, he is working on rare forms of sensoryneural deafness, using a combination of genomic, molecular and cellular approaches, in the zebrafish, in order to elucidate the function of genes important for the audition in human.
Some publications (see below) about the study of Myo7a gene:
- Article describing the identification and the characterization of the first zebrafish model of human deafness
- Study of the regulation of Myo7a gene expression in zebrafish
Resources & publications
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Journal (source)Cell Death Discov
Kremen1-induced cell death is regulated by homo- and heterodimerization.
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Journal (source)Curr. Opin. Neurobiol.
Cortical developmental death: selected to survive or fated to die.
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Journal (source)Evodevo
Neuronal fate specification by the Dbx1 transcription factor is linked to the...
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Journal (source)Curr. Biol.
Migration Speed of Cajal-Retzius Cells Modulated by Vesicular Trafficking Con...
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Journal (source)PLoS ONE
Dbx1-expressing cells are necessary for the survival of the mammalian anterio...
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Journal (source)PLoS Biol.
A novel role for Dbx1-derived Cajal-Retzius cells in early regionalization of...
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Journal (source)Cell Death Differ.
Kremen1 and Dickkopf1 control cell survival in a Wnt-independent manner.
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Journal (source)bioRxiv
Functional characterization of RELN missense mutations involved in recessive ...
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Journal (source)Development
The multiple facets of Cajal-Retzius neurons.
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Journal (source)Development
Single-cell transcriptomics of the early developing mouse cerebral cortex dis...
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Journal (source)Dev Cell
Repurposing of the multiciliation gene regulatory network in fate specificati...
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Journal (source)Curr Opin Neurobiol
Cajal-retzius cells: Recent advances in identity and function
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Journal (source)J Comp Neurol
Diversity within olfactory sensory derivatives revealed by the contribution o...
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Journal (source)Dev Cell
Repurposing of the multiciliation gene regulatory network in fate specificati...
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Journal (source)Dev Cell
Repurposing of the multiciliation gene regulatory network in fate specificati...
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Journal (source)J Clin Invest
De novo monoallelic Reelin missense variants act in a dominant-negative manne...
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Journal (source)J Clin Invest
De novo monoallelic Reelin missense variants cause dominant neuronal migratio...
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Journal (source)Development
Differential contribution of P73+ Cajal-Retzius cells and Reelin to cortical ...