Journal article
Mice lacking Dfna5 show a diverging number of cochlear fourth row outer hair cells
Neurobiology of disease, Vol.19(3), pp.386-399
2005
DOI: 10.1016/j.nbd.2005.01.019
PMID: 16023581
Abstract
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 Auditory Brainstem Response (ABR) measurements were performed at different ages in two genetic backgrounds (C57Bl/6J and CBA/Ca), but no differences between Dfna5−/− and Dfna5+/+ mice could be demonstrated. Morphological studies demonstrated significant differences in the number of fourth row outer hair cells between Dfna5−/− mice and their wild-type littermates. These results were obtained in both genetic backgrounds, albeit with opposite effects. In contrast to the results obtained in Dfna5−/− zebrafish, we did not observe different UDP-glucose dehydrogenase and hyaluronic acid levels in Dfna5−/− mice when compared to Dfna5+/+ mice.
Details
- Title: Subtitle
- Mice lacking Dfna5 show a diverging number of cochlear fourth row outer hair cells
- Creators
- Lut Van Laer - Department of Medical Genetics, University of Antwerp, Campus Drie Eiken, Universiteitsplein 1, B-2610 Antwerp, BelgiumMarkus Pfister - Hals-Nasen-Ohrenklinik, Universität of Tübingen, Tübingen, GermanySofie Thys - Department of Medical Genetics, University of Antwerp, Campus Drie Eiken, Universiteitsplein 1, B-2610 Antwerp, BelgiumKaren Vrijens - Department of Medical Genetics, University of Antwerp, Campus Drie Eiken, Universiteitsplein 1, B-2610 Antwerp, BelgiumMarcus Mueller - Hals-Nasen-Ohrenklinik, Universität of Tübingen, Tübingen, GermanyLieve Umans - Experimental Genetics Group, University of Leuven, Leuven, BelgiumLutgarde Serneels - Experimental Genetics Group, University of Leuven, Leuven, BelgiumLuc Van Nassauw - Laboratory of Cell Biology and Histology, University of Antwerp, Campus Middelheim, Antwerp, BelgiumFrank Kooy - Department of Medical Genetics, University of Antwerp, Campus Drie Eiken, Universiteitsplein 1, B-2610 Antwerp, BelgiumRichard J.H Smith - Molecular Otolaryngology Research Laboratories, University of Iowa, Iowa City, IA 52242-1182, USAJean-Pierre Timmermans - Laboratory of Cell Biology and Histology, University of Antwerp, Campus Middelheim, Antwerp, BelgiumFred Van Leuven - Experimental Genetics Group, University of Leuven, Leuven, BelgiumGuy Van Camp - Department of Medical Genetics, University of Antwerp, Campus Drie Eiken, Universiteitsplein 1, B-2610 Antwerp, Belgium
- Resource Type
- Journal article
- Publication Details
- Neurobiology of disease, Vol.19(3), pp.386-399
- DOI
- 10.1016/j.nbd.2005.01.019
- PMID
- 16023581
- NLM abbreviation
- Neurobiol Dis
- ISSN
- 0969-9961
- eISSN
- 1095-953X
- Publisher
- Elsevier Inc
- Language
- English
- Date published
- 2005
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
- Record Identifier
- 9984006442902771
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