Cochlin expression in vestibular endorgans obtained from patients with Meniere's disease
Cochlin expression in vestibular endorgans obtained from patients with Meniere's disease
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DOI：
10.1007/s00441-012-1481-x
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发表时间：
2012-11-01
影响因子：
3.6
通讯作者：
Ishiyama, Gail
中科院分区：
文献类型：
--
作者：
Calzada, Audrey P.;Lopez, Ivan A.;Ishiyama, Gail
cochlin及其相关的基底膜蛋白（胶原蛋白IV，胶原蛋白II，层粘连蛋白-β 2，巢蛋白-1）的分布进行了评估与梅尼埃病的受试者的前庭终器，并与正常标本进行比较。还研究了来自梅尼埃病标本的前庭终器中Cochlin mRNA的表达。标本来自梅尼埃病患者和正在接受消融性腰椎切除术的患者。对照标本均来自尸检标本，记录正常的听前庭功能，并从患者接受听神经瘤切除术。正常对照组壶腹嵴间质和椭圆囊斑内呈均匀分布。在梅尼埃的标本，cochlin免疫反应性显着增加，这是与cochlin mRNA表达的增加，所示的实时逆转录聚合酶链反应。胶原IV和层粘连蛋白-β 2免疫反应性在梅尼埃标本中显著降低。巢蛋白-1和II型胶原蛋白免疫反应性与正常样本相比，在梅尼埃的标本是不变的。Cochlin上调与遗传性听前庭病DFNA 9有关。在梅尼埃病中cochlin的表达增加和胶原IV和层粘连蛋白的表达减少提示cochlin的过表达有助于在这种疾病中观察到的功能失调的内耳稳态。
The distribution of cochlin and its associated basement membrane proteins (collagen IV, collagen II, laminin-beta 2, and nidogen-1) were evaluated in the vestibular endorgans of subjects with Meniere's disease and compared with normal specimens. Cochlin mRNA expression in vestibular endorgans from Meniere's disease specimens was also investigated. Specimens were obtained from patients who had Meniere's disease and who were undergoing ablative labyrinthectomy. Control specimens were obtained both from autopsy specimens with documented normal audiovestibular function and from patients undergoing labyrinthectomy for acoustic neuroma excision. In the normal control specimens, cochlin immunoreactivity was found evenly distributed in the stroma of the cristae ampullaris and maculae of the utricle. In Meniere's specimens, cochlin immunoreactivity was markedly increased; this was associated with an increase in cochlin mRNA expression as shown by real-time reverse transcription with the polymerase chain reaction. Collagen IV and laminin-beta 2 immunoreactivity was significantly decreased in Meniere's specimens. Nidogen-1 and collagen II immunoreactivity was unchanged in Meniere's specimens when compared with normal samples. Cochlin upregulation has been implicated in the hereditary audiovestibulopathy, DFNA9. The increased expression of cochlin and decreased expression of collagen IV and laminin in Meniere's disease are suggestive that the overexpression of cochlin contributes to the dysfunctional inner ear homeostasis seen in this disease.