Article
Mutation of the TBCE gene causes disturbance of microtubules in the auditory nerve and cochlear outer hair cell degeneration accompanied by progressive hearing loss in the pmn/pmn mouse.
Experimental neurology - 1 Dec 2013
Rak Kristen, Frenz Silke, Radeloff Andreas, Groh Janos, Jablonka Sibylle, Martini Rudolf, Hagen Rudolf, Mlynski Robert
Abstract excerpt
The progressive motor neuronopathy (pmn/pmn) mouse, an animal model for a fast developing human motor neuron disorder, is additionally characterized by simultaneous progressive sensorineural hearing loss. The gene defect in the pmn/pmn mouse is localized to a missense mutation in the tubulin-specific chaperone E (TBCE) gene on mouse chromosome 13, which is one of the five tubulin-specific chaperons involved in...
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