Anti-apoptotic gene Bcl2 is required for stapes development and hearing

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dc.contributor.authorCarpinelli, Marina
dc.contributor.authorWise, Andrew
dc.contributor.authorArhatari, Benedicta
dc.contributor.authorBouillet, Phillipe
dc.contributor.authorManji, Shehnaaz
dc.contributor.authorManning, Michael
dc.contributor.authorCooray, Anne
dc.contributor.authorBurt, Rachel
dc.date.accessioned2013-10-04T00:15:05Z
dc.date.available2013-10-04T00:15:05Z
dc.date.issued2012-08-09
dc.descriptionPublisher's link to article: http://www.nature.com/cddis/journal/v3/n8/full/cddis2012100a.htmlen_US
dc.description.abstractIn this paper we describe novel and specific roles for the apoptotic regulators Bcl2 and Bim in hearing and stapes development. Bcl2 is anti-apoptotic while Bim is pro-apoptotic. Characterization of the auditory systems of mice deficient for these molecules revealed that Bcl2 / mice suffered severe hearing loss. This was conductive in nature and did not affect sensory cells of the inner ear, with cochlear hair cells and neurons present and functional. Bcl2 / mice were found to have a malformed, often monocrural, porous stapes (the small stirrup-shaped bone of the middle ear), but a normally shaped malleus and incus. The deformed stapes was discontinuous with the incus and sometimes fused to the temporal bones. The defect was completely rescued in Bcl2 / Bim / mice and partially rescued in Bcl2 / Bimþ/ mice, which displayed high-frequency hearing loss and thickening of the stapes anterior crus. The Bcl2 / defect arose in utero before or during the cartilage stage of stapes development. These results implicate Bcl2 and Bim in regulating survival of second pharyngeal arch or neural crest cells that give rise to the stapes during embryonic development.en_US
dc.description.sponsorshipHearing CRC, established and supported under the Australian Government’s Cooperative Research Centres Program, the Victorian State Government’s Operational Infrastructure Support Program and the Australian Government’s NHMRC IRIISS. MRC was supported by the Garnet Passe and Rodney Williams Memorial Foundation. AKW was supported by the National Institutes of Health (HHS-N-263- 2007-00053-C). BDA acknowledges the support of the Australian Research Council through the Centre of Excellence for Coherent X-ray science.en_US
dc.identifier.citationCarpinelli, M. R., Wise, A., Arhatari, B., Bouillet, P., Manji, S., Manning, M., . . . Burt, R. A. (2012). Anti-apoptotic gene Bcl2 is required for stapes development and hearing. Cell Death and Disease, e362. doi: 10.1038/cddis.2012.100en_US
dc.identifier.otherDOI:10.1038/cddis.2012.100
dc.identifier.urihttp://repository.bionicsinstitute.org:8080/handle/123456789/50
dc.language.isoenen_US
dc.publisherMacmillan Publishers Limiteden_US
dc.subjectHearingen_US
dc.subjectMouseen_US
dc.subjectGeneen_US
dc.subjectStapesen_US
dc.subjectHearing Developmenten_US
dc.subjectApoptosisen_US
dc.subjectBCL2en_US
dc.subjectHair Cellsen_US
dc.titleAnti-apoptotic gene Bcl2 is required for stapes development and hearingen_US
dc.typeArticleen_US
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