Article
A mouse model for human deafness DFNB22 reveals that hearing impairment is due to a loss of inner hair cell stimulation.
Proceedings of the National Academy of Sciences of the United States of America - 20 Nov 2012
Lukashkin Andrei N, Legan P Kevin, Weddell Thomas D, Lukashkina Victoria A, Goodyear Richard J, Welstead Lindsey J, Petit Christine, Russell Ian J, Richardson Guy P
Abstract excerpt
The gene causative for the human nonsyndromic recessive form of deafness DFNB22 encodes otoancorin, a 120-kDa inner ear-specific protein that is expressed on the surface of the spiral limbus in the cochlea. Gene targeting in ES cells was used to create an EGFP knock-in, otoancorin KO (Otoa(EGFP/EGFP)) mouse. In the Otoa(EGFP/EGFP) mouse, the tectorial membrane (TM), a ribbon-like strip of ECM that is normally...
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