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.
Expression of Myo7a in sensory hair cells (inner ear and neuromasts) of zebrafish. Left: in situ hybridization. Right: transgenic zebrafish expressing EGFP fluorescent protein under the control of a Myo7a regulatory element.
©
Sylvain Ernest
Expression of Myo7aa-mCherry fusion protein (red) in sensory hair cells of zebrafish inner ear. Labeling of actin rich hair bundles (green).
©
Sylvain Ernest
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