In the recessive
deafness model, gene therapy with TMC1 restored the ability of sensory hair cells to respond to sound — producing a measurable electrical current — and also restored activity in the auditory portion of the brainstem.
In the dominant
deafness model, gene therapy with a related gene, TMC2, was successful at the cellular and brain level, and partially successful at restoring actual hearing in the startle test.
However, Holt's study also showed that gene therapy with TMC2 could compensate for loss of a functional TMC1 gene, restoring hearing in the recessive
deafness model and partial hearing in the dominant
deafness model.
Not exact matches
The researchers performed state - of - the - art next generation sequencing (NGS) on a
model of human
deafness to identify the critical lncRNAs.
So far, gene therapy attempts have only resulted in partial improvements of hearing in mouse
models of specific human
deafness forms that did not include severe anomalies in hair cell structure.
Researchers from the Eaton - Peabody Laboratories of the Massachusetts Eye and Ear and Harvard Medical School have created a new mouse
model in which by expressing a gene in the inner ear hair cells — the sensory cells that detect sound and sense balance — protects the mice from age - related hearing loss (ARHL) and noise - induced hearing loss (NIHL), the two most common forms of
deafness.
Otoferlin's size has precluded rescue experiments in which a modified mRNA for otoferlin is transfected into an animal
model to replace a suppressed or knocked - down otoferlin gene causing
deafness.
«NIH is committed to making knockout mouse
models more widely accessible to the biomedical research community,» said James Battey, M.D., Ph.D., director of the National Institute on
Deafness and Other Communication Disorders (NIDCD), who is also the co-chair of the Trans - NIH Mouse Initiative.
Hennebold is attempting to create these «knockout»
models for human diseases like hereditary blindness and
deafness, among other disorders.
Yet another primary interest is in the genetics of hearing and
deafness disorders using mouse
models of human
deafness disorders and genome - wide association studies in age - related hearing impairment.
The U.S. Department of Transportation is committed to being a
model employer of a diverse workforce that includes people with disabilities, especially those with targeted disabilities (blindness,
deafness, partial and full paralysis, missing extremities, dwarfism, epilepsy, intellectual disabilities, and psychiatric disabilities).