University of Houston Researcher Builds New Model to Examine Usher Syndrome

An important component in the journey to developing treatments for Usher syndrome is having an animal model to understand what is happening on a cellular and genetic basis. With Usher syndrome specifically, it has been historically challenging to produce a model that has both the hearing loss and visual problems reported in patients. Dr. Muna Naash at the University of Houston reported successful design and generation of a model with a specific gene mutation found in USH2A (c.2299delG) that shows retinal degeneration. 

What this means for Usher syndrome: Having a model that reproduces the USH2A retinal phenotype is a solid starting point for designing future therapeutic interventions. It also allows Naash’s team to study the mechanism of the disease more thoroughly.

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