BackgroundMyosin VIIA (MyoVIIA) is an unconventional myosin necessary for vertebrate audition –. Human auditory transduction occurs in sensory hair cells with a staircase-like arrangement of apical protrusions called stereocilia. In these hair cells, MyoVIIA maintains stereocilia organization . Severe mutations in the Drosophila MyoVIIA orthologue, crinkled (ck), are semi-lethal and lead to deafness by disrupting antennal auditory organ (Johnston's Organ, JO) organization . ck/MyoVIIA mutations result in apical detachment of auditory transduction units (scolopidia) from the cuticle that transmits antennal vibrations as mechanical stimuli to JO.
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机译:背景肌球蛋白VIIA(MyoVIIA)是脊椎动物试听所必需的非常规肌球蛋白–。人的听觉传导发生在感觉性毛细胞中,其顶端呈阶梯状排列,称为立体纤毛。在这些毛细胞中,MyoVIIA维持纤毛组织。果蝇MyoVIIA直系同源基因中的严重突变是皱纹(ck),具有半致命性,并通过破坏触觉听觉器官(Johnston's Organ,JO)导致耳聋。 ck / MyoVIIA突变导致表皮的听觉转导单元(虹膜)根尖分离,从而将触角振动作为机械刺激传递给JO。
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