Demyelination and Na+Channel Redistribution Underlie Auditory and Vestibular Dysfunction in PMP22-Null Mice
Jeong Han Lee,
Seojin Park,
Maria C. Perez-Flores
et al.
Abstract:Altered expression of peripheral myelin protein 22 (PMP22) results in demyelinating peripheral neuropathy. PMP22 exhibits a highly restricted tissue distribution with marked expression in the myelinating Schwann cells of peripheral nerves. Auditory and vestibular Schwann cells and the afferent neurons also express PMP22, suggesting a unique role in hearing and balancing. Indeed, neuropathic patients diagnosed with PMP22-linked hereditary neuropathies often present with auditory and balance deficits, an underst… Show more
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