Article
Mice lacking Dfna5 show a diverging number of cochlear fourth row outer hair cells.
Neurobiology of disease - 1 Aug 2005
Van Laer Lut, Pfister Markus, Thys Sofie, Vrijens Karen, Mueller Marcus, Umans Lieve, Serneels Lutgarde, Van Nassauw Luc, Kooy Frank, Smith Richard J H, Timmermans Jean-Pierre, Van Leuven Fred, Van Camp Guy
Abstract excerpt
A complex mutation in DFNA5, resulting in exon 8 skipping, causes autosomal dominant hearing impairment, which starts in the high frequencies between 5 and 15 years of age and progressively affects all frequencies. To study its function in vivo, Dfna5 knockout mice were generated through the deletion of exon 8, simultaneously mimicking the human mutation. To test the hearing impairment, frequency-specific...
Topics
- Animals
- Blotting, Western
- Cochlea
- Disease Models, Animal
- Evoked Potentials, Auditory, Brain Stem
- Genotype
- Hair Cells, Auditory, Outer
- Hearing Loss
- Hyaluronic Acid
- Mice