Swartz D J, Santi P A
University of Minnesota Medical School, Otolaryngology and Neurosciences, Minneapolis 55455, USA.
Hear Res. 1997 Jul;109(1-2):92-101. doi: 10.1016/s0378-5955(97)00054-3.
Keratan sulfate (KS) was immunolocalized in the chinchilla cochlea and vestibular system using indirect immunohistochemistry and a monoclonal antibody (clone 5-D-4) directed against a proteoglycan core antigen. As a positive control, anti-KS Mab reactivity was found in the pericellular matrix and lacuna walls of temporal bone osteocytes. In the cochlea, anti-KS Mab reactivity was abundant in the basal cell layer of the stria vascularis and in the marginal band and Hensen's stripe of the tectorial membrane. Less anti-KS Mab reactivity was present in the cover net, Hardesty's membrane and the upper fibrous zone of the limbal layer of the tectorial membrane. In the vestibular system, anti-KS Mab reactivity was immunolocalized to a portion of the epithelium overlying the cupula of the crista ampullaris, in the apical surface of crista ampullaris epithelium, crista ampullaris stereocilia and in the otoconia. Elucidating the distribution of KS in the cochlea will improve our understanding of cochlear anatomy and is a first step toward understanding the etiology of hearing loss observed in diseases involving KS metabolism, namely, mucopolysaccharidosis type IV (Morquio's syndrome).
使用间接免疫组织化学和针对蛋白聚糖核心抗原的单克隆抗体(克隆5-D-4),对硫酸角质素(KS)在龙猫耳蜗和前庭系统中进行免疫定位。作为阳性对照,在颞骨骨细胞的细胞周基质和腔隙壁中发现了抗KS单克隆抗体反应性。在耳蜗中,抗KS单克隆抗体反应性在血管纹的基底细胞层以及盖膜的边缘带和亨森带中丰富。在盖网、哈德斯蒂膜和盖膜边缘层的上部纤维区中存在较少的抗KS单克隆抗体反应性。在前庭系统中,抗KS单克隆抗体反应性免疫定位于壶腹嵴顶帽上方的部分上皮、壶腹嵴上皮的顶端表面、壶腹嵴静纤毛和耳石中。阐明KS在耳蜗中的分布将增进我们对耳蜗解剖结构的理解,并且是迈向理解在涉及KS代谢的疾病(即IV型粘多糖贮积症(莫尔基奥综合征))中观察到的听力损失病因的第一步。