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[螺旋韧带纤维细胞在内耳代谢调节中的干预作用]

[Intervention of spiral ligament fibrocytes in the metabolic regulation of the inner ear].

作者信息

García Berrocal José Ramón, Méndez-Benegassi Iván, Martí Cristina, Ramírez Camacho Rafael

机构信息

Servicio de Otorrinolaringología. Hospital Universitario Puerta de Hierro. Universidad Autónoma de Madrid. Madrid. España.

出版信息

Acta Otorrinolaringol Esp. 2008 Dec;59(10):494-9.

Abstract

Maintenance of the K(+) gradient between endolymph and perilymph is essential for normal hearing and depends primarily on the activity of the stria vascularis. Abundant Na-K-ATPase in marginal strial cells provides a pumping mechanism for preserving the K(+) level of the endolymph and consequently, the endocochlear potential. Fibrocytes in the lateral wall of the cochlea supply K(+) to the strial pump, via gap junctions, by recycling back into the stria the ions that efflux from the scala media during auditory transduction. The lateral wall of the cochlea encloses five types of fibrocytes, differentiated by their location, structural features and content of enzymes mediating or energizing ion transport. The disruption of the gap junction bonds by connexin mutations and other pathologies leads to an interruption of K(+) recirculation pathways. The expression of cochlin and otoraplin, proteins that participate in structural or regulatory functions in the inner ear, suggests more diversity and complexity of the mesenchymal tissues than envisioned previously. The presence of otospiralin, a novel protein found in fibrocytes of spiral limbus, spiral ligament and subepithelial regions of the vestibule, represent a critical finding since that protein has been shown to be essential for the survival of the hair cells and supporting cells of the inner ear. A more profound knowledge and understanding of the function of inner ear fibrocytes will provide a new and promising aetiopathogenic approach to the treatment of inner ear disorders.

摘要

维持内淋巴和外淋巴之间的钾离子梯度对于正常听力至关重要,并且主要依赖于血管纹的活性。边缘血管纹细胞中丰富的钠钾ATP酶提供了一种泵机制,用于维持内淋巴中的钾离子水平,进而维持内耳蜗电位。耳蜗外侧壁的纤维细胞通过缝隙连接,将听觉转导过程中从蜗管中流出的离子再循环回血管纹,从而为血管纹泵供应钾离子。耳蜗外侧壁包含五种类型的纤维细胞,根据它们的位置、结构特征以及介导或促进离子转运的酶的含量进行区分。连接蛋白突变和其他病理状况导致缝隙连接键的破坏,进而导致钾离子再循环途径中断。耳蜗素和耳螺旋素的表达表明内耳间充质组织比之前预想的具有更多样性和复杂性,这两种蛋白质在内耳中参与结构或调节功能。耳螺旋蛋白是在螺旋缘、螺旋韧带和前庭上皮下区域的纤维细胞中发现的一种新型蛋白质,它的存在是一个关键发现,因为该蛋白质已被证明对内耳毛细胞和支持细胞的存活至关重要。对内耳纤维细胞功能更深入的了解将为内耳疾病的治疗提供一种新的、有前景的病因学治疗方法。

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