Bionics Institute Research Online >
Bionic Hearing >
Bionic Hearing Research Publications >
|Title: ||Anti-apoptotic gene Bcl2 is required for stapes development and hearing|
|Authors: ||Carpinelli, Marina|
|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|
|Appears in Collections:||Bionic Hearing Research Publications|
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.