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Title: Anti-apoptotic gene Bcl2 is required for stapes development and hearing
Authors: Carpinelli, Marina
Wise, Andrew
Arhatari, Benedicta
Bouillet, Phillipe
Manji, Shehnaaz
Manning, Michael
Cooray, Anne
Burt, Rachel
Keywords: Hearing
Hearing Development
Hair Cells
Issue Date: 9-Aug-2012
Publisher: Macmillan Publishers Limited
Citation: Carpinelli, 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.2012.100
Abstract: In 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.
Description: Publisher's link to article: http://www.nature.com/cddis/journal/v3/n8/full/cddis2012100a.html
URI: http://repository.bionicsinstitute.org:8080/handle/123456789/50
Appears in Collections:Bionic Hearing Research Publications

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