A deafness mutation isolates a second role for the tectorial membrane in hearing

Legan, P Kevin, Lukashkina, Victoria A, Goodyear, Richard J, Lukashkin, Andrei N, Verhoeven, Kristien, Van Camp, Guy, Russell, Ian J and Richardson, Guy P (2005) A deafness mutation isolates a second role for the tectorial membrane in hearing. Nature Neuroscience, 8. pp. 1035-1042. ISSN 1097-6256

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a-tectorin (encoded by Tecta) is a component of the tectorial membrane, an extracellular matrix of the cochlea. In humans, the Y1870C missense mutation in TECTA causes a 50- to 80-dB hearing loss. In transgenic mice with the Y1870C mutation in Tecta, the tectorial membrane's matrix structure is disrupted, and its adhesion zone is reduced in thickness. These abnormalities do not seriously influence the tectorial membrane's known role in ensuring that cochlear feedback is optimal, because the sensitivity and frequency tuning of the mechanical responses of the cochlea are little changed. However, neural thresholds are elevated, neural tuning is broadened, and a sharp decrease in sensitivity is seen at the tip of the neural tuning curve. Thus, using TectaY1870C/+ mice, we have genetically isolated a second major role for the tectorial membrane in hearing: it enables the motion of the basilar membrane to optimally drive the inner hair cells at their best frequency.

Item Type: Article
Additional Information: GPR and IJR directed the research and were joint corresponding authors. The paper describes a mouse model for a missense mutation that causes deafness in humans and provides the first experimental evidence that the tectorial membrane plays a critical role in tuning the responses of the cochlear inner hair cells.
Schools and Departments: School of Life Sciences > Neuroscience
Depositing User: Kevin Legan
Date Deposited: 06 Feb 2012 19:34
Last Modified: 30 Nov 2012 17:03
URI: http://sro.sussex.ac.uk/id/eprint/21352
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