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Publication : β-Actin and fascin-2 cooperate to maintain stereocilia length.

First Author  Perrin BJ Year  2013
Journal  J Neurosci Volume  33
Issue  19 Pages  8114-21
PubMed ID  23658152 Mgi Jnum  J:197137
Mgi Id  MGI:5490924 Doi  10.1523/JNEUROSCI.0238-13.2013
Citation  Perrin BJ, et al. (2013) beta-Actin and Fascin-2 Cooperate to Maintain Stereocilia Length. J Neurosci 33(19):8114-21
abstractText  Stereocilia are actin-based protrusions on auditory sensory hair cells that are deflected by sound waves to initiate the conversion of mechanical energy to neuronal signals. Stereocilia maintenance is essential because auditory hair cells are not renewed in mammals. This process requires both beta-actin and gamma-actin as knock-out mice lacking either isoform develop distinct stereocilia pathology during aging. In addition, stereocilia integrity may hinge on immobilizing actin, which outside of a small region at stereocilia tips turns over with a very slow, months-long half-life. Here, we establish that beta-actin and the actin crosslinking protein fascin-2 cooperate to maintain stereocilia length and auditory function. We observed that mice expressing mutant fascin-2 (p.R109H) or mice lacking beta-actin share a common phenotype including progressive, high-frequency hearing loss together with shortening of a defined subset of stereocilia in the hair cell bundle. Fascin-2 binds beta-actin and gamma-actin filaments with similar affinity in vitro and fascin-2 does not depend on beta-actin for localization in vivo. Nevertheless, double-mutant mice lacking beta-actin and expressing fascin-2 p.R109H have a more severe phenotype suggesting that each protein has a different function in a common stereocilia maintenance pathway. Because the fascin-2 p.R109H mutant binds but fails to efficiently crosslink actin filaments, we propose that fascin-2 crosslinks function to slow actin depolymerization at stereocilia tips to maintain stereocilia length.
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