Article
Pejvakin, a Candidate Stereociliary Rootlet Protein, Regulates Hair Cell Function in a Cell-Autonomous Manner.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 29 Mar 2017
Kazmierczak Marcin, Kazmierczak Piotr, Peng Anthony W, Harris Suzan L, Shah Prahar, Puel Jean-Luc, Lenoir Marc, Franco Santos J, Schwander Martin
Abstract excerpt
Mutations in the Pejvakin (PJVK) gene are thought to cause auditory neuropathy and hearing loss of cochlear origin by affecting noise-induced peroxisome proliferation in auditory hair cells and neurons. Here we demonstrate that loss of pejvakin in hair cells, but not in neurons, causes profound hearing loss and outer hair cell degeneration in mice. Pejvakin binds to and colocalizes with the rootlet component...
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