Cochlin expression in vestibular endorgans obtained from patients with Meniere's disease

被引:32
作者
Calzada, Audrey P. [2 ]
Lopez, Ivan A. [2 ]
Parrazal, Luis Beltran [3 ]
Ishiyama, Akira [2 ]
Ishiyama, Gail [1 ,4 ]
机构
[1] Univ Calif Los Angeles, Dept Neurol, Reed Neurol Res Ctr, Los Angeles, CA 90095 USA
[2] Univ Calif Los Angeles, David Geffen Sch Med, Dept Head & Neck Surg, Los Angeles, CA 90095 USA
[3] Univ Veracruzana, Ctr Cerebral Invest, Veracruz, Veracruz, Mexico
[4] Univ Calif Los Angeles, David Geffen Sch Med, Dept Neurol, Los Angeles, CA 90095 USA
基金
美国国家卫生研究院;
关键词
Cochlin; Basement membrane; Meniere's disease; Inner ear; Vestibular endorgans; Human; INNER-EAR; BASEMENT-MEMBRANE; MESSENGER-RNA; EXTRACELLULAR-MATRIX; STRIA VASCULARIS; II COLLAGEN; MOUSE MODEL; GENE; LOCALIZATION; DEAFNESS;
D O I
10.1007/s00441-012-1481-x
中图分类号
Q2 [细胞生物学];
学科分类号
071013 [干细胞生物学];
摘要
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.
引用
收藏
页码:373 / 384
页数:12
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