Hearing myosin

Two other myosin types have been implicated in hearing and vestibular function [62]. The defect in the Snell s waltzer mouse was found to be a mutation in a myosin VI gene that produces degeneration of the cochlea and vestibular apparatus. Myosin VI is localized to the cuticular plate of the hair cell under stereocilia. Similarly, mutations in a myosin VII gene are responsible for the shaker-1 mouse and several human genetic deafness disorders. This myosin, myosin Vila, is found in a band near the base of the stereocilia distinct from distributions of myosin ip and myosin VI. 

A characteristic is the appearance of "nemaline bodies" or thickened Z-discs containing Z-disc proteins. Some hereditary diseases involve nonmuscle myosins. Among these is Usher syndrome, the commonest cause of deaf-blindness. The disease, which results from a defect in the myosin VIA gene, typically causes impairment of hearing and retinitis pigmentosa (Chapter 23).hh... 

Myosin, an essential protein component of contractile muscle together with actin. Myosins are eukaryotic actin-dependent molecular motors with high importance for a wide range of functions such as muscle contraction, vision, hearing, cell motility, and host cell invasion of apicomplexan parasites. Myosin forms almost entirely the... 

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