Defects in the MITFmi/mi apical surface are associated with a failure of outer segment elongation

Defects in the MITFmi/mi apical surface are associated with a failure of outer segment elongation
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DOI:
10.1006/exer.2001.1048
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发表时间:
2001-09-01
影响因子:
3.4
通讯作者:
Barnstable, CJ
Barnstable, CJ
中科院分区:
医学3区
文献类型:
--
作者:
Bumsted, KM;Rizzolo, LJ;Barnstable, CJ

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MITFmi/mi小鼠中MITF功能的丧失不仅影响RPE分化,还影响视杆细胞外节的发育。我们的数据表明,在细胞膜和沿着ONL/RPE边界的开发MITFmi/mi杆光感受器和杆外段形态发生启动检测到视蛋白免疫反应。尽管与外节相关的分子继续表达,但外节没有伸长并形成垂直于RPE的堆叠组织盘。MITFmi/mi RPE也未能形成顶端微绒毛,并且缺乏野生型组织中所见的磷蛋白ezrin的顶端网络。MITFmi/mi RPE基底表面组织松散,并保留ezrin标记,表明一定程度的分化。在我们的数据中看到的相关性表明,在RPE顶端域形成的失败和杆外段伸长的缺乏之间可能存在联系。(C)北京:科学出版社.
The loss of MITF function in the MITFmi/mi mouse affects not only RPE differentiation, but also the development of rod photoreceptor outer segments. Our data indicate that opsin immunoreactivity is detected in the cell membrane and along the ONL/RPE border of developing MITFmi/mi rod photoreceptors and that rod outer segment morphogenesis is initiated. Although molecules associated with the outer segment continued to be expressed, outer segments did not elongate and develop stacked organized discs perpendicular to the RPE. The MITFmi/mi RPE also failed to form apical microvilli and lacked the apical network of the phosphoprotein ezrin seen in wild type tissue. The MITFmi/mi RPE basal surface was loosely organized and retained ezrin labelling which indicated some degree of differentiation. The correlation seen in our data suggest that there may be a link between the failure of the RPE apical domain to form and lack of rod outer segment elongation. (C) 2001 Academic Press.