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首页> 外文期刊>The European Journal of Neuroscience >A novel stereocilia defect in sensory hair cells of the deaf mouse mutant Tasmanian devil.
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A novel stereocilia defect in sensory hair cells of the deaf mouse mutant Tasmanian devil.

机译：聋小鼠突变塔斯马尼亚恶魔的感觉毛细胞中的新型立体纤毛缺陷。

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Stereocilia are specialized actin-filled, finger-like processes arrayed in rows of graded heights to form a crescent or W-shape on the apical surface of sensory hair cells. The stereocilia are deflected by the vibration of sound, which opens transduction channels and allows an influx of ions to depolarize the hair cell, in turn triggering synaptic activity. The specialized morphology and organization of the stereocilia bundle is crucial in the process of sensory transduction in the inner ear. However, we know little about the development of stereocilia in the mouse and few molecules that are involved in stereocilia maturation are known. We describe here a new mouse mutant with abnormal stereocilia development. The Tasmanian devil (tde) mouse mutation arose by insertional mutagenesis and has been mapped to the middle of chromosome 5. Homozygotes show head-tossing and circling and have raised thresholds for cochlear nerve responses to sound. The gross morphology of the inner ear was normal, but the stereocilia of cochlear and vestibular hair cells are abnormally thin, and they become progressively disorganized with increasing age. Ultimately, the hair cells die. This is the first report of a mutant showing thin stereocilia. The association of thin stereocilia with cochlear dysfunction emphasizes the critical role of stereocilia in auditory transduction, and the discovery of the Tasmanian devil mutant provides a resource for the identification of an essential molecule in hair cell function.
机译：立体毛虫是专门的肌动蛋白填充，手指状过程，排列成高低梯度行，在感觉毛细胞的顶表面形成月牙形或W形。立体声振动通过声音的振动而偏转，这打开了转导通道并允许离子流入使毛细胞去极化，从而触发突触活动。立体纤毛束的特殊形态和组织在内耳感觉传导过程中至关重要。但是，我们对小鼠中的纤毛虫的发育了解甚少，并且与纤毛虫成熟有关的分子很少。我们在这里描述了一个新的具有异常的纤毛发育的小鼠突变体。塔斯马尼亚恶魔（tde）小鼠突变是由插入诱变引起的，已定位到5号染色体的中部。纯合子表现出头顶摆动和盘旋状态，并且耳蜗神经对声音的反应阈值升高。内耳的总体形态正常，但是耳蜗和前庭毛细胞的立体纤毛异常稀薄，并且随着年龄的增长逐渐变得混乱。最终，毛细胞死亡。这是一个突变体显示稀薄的纤毛的首次报道。纤细的纤毛纤毛与耳蜗功能障碍的关联强调了纤毛纤毛在听觉传导中的关键作用，塔斯马尼亚恶魔突变体的发现为鉴定毛细胞功能中的必需分子提供了资源。

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