Connexin 26 in human fetal development of the inner ear
Connexin 26 in human fetal development of the inner ear
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DOI：
10.1016/s0378-5955(01)00310-0
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发表时间：
2001-10-01
期刊：
影响因子：
2.8
通讯作者：
Schrott-Fischer, A
中科院分区：
文献类型：
--
作者：
Kammen-Jolly, K;Ichiki, H;Schrott-Fischer, A
间隙连接专门用于细胞间通讯，理论上可提供一种手段（上皮和结缔组织间隙连接系统），通过该手段可以运输液体和离子以维持高水平的内淋巴 K+ [Kikuchi 等人，1994。Acta Otolaryngol。 114, 520-528]在内耳中。这些间隙连接的主要成分是连接蛋白 26 (Cx26)，这是一种由 GJB2 基因编码的蛋白质，存在于上皮细胞和结缔组织细胞中。研究表明，50% 的常染色体隐性遗传性非综合征性听力损失患者中存在 Cx26 突变。在本研究中，我们记录了 Cx26 在人类胎儿耳蜗妊娠各个阶段（第 11-31 周）的出现和分布特征。 Cx26 分布的比较模式也出现在成熟大鼠中。死后 2 小时内将耳蜗固定在 4% 多聚甲醛中。使用针对合成肽且对应于氨基酸 108-122 的兔多克隆抗体进行免疫组织化学研究。将样品装入石蜡切片中。结果显示，Cx26 样免疫反应性在产前 11 周时就很明显，并在妊娠 31 周期间保持高强度的反应性。这种反应性的出现似乎与发育和组织学成熟的开始同时进行调节，并提供功能维持。在人类胎儿耳蜗中，Cx26 样免疫反应性分布在胎儿第 20 周时类似于成人模式。在第 31 周形态发育完成时，反应性似乎达到了成人的分布情况。详细描述和讨论了 Cx26 分布模式。 (C) 2001 Elsevier Science B.V. 保留所有权利。
Specialized for intercellular communication, gap junctions have been theorized to provide a means (the epithelial and connective tissue gap junction systems) by which fluid and ions might be transported for maintenance of high levels of endolymphatic K+ [Kikuchi et al., 1994. Acta Otolaryngol. 114, 520-528] in the inner ear. A primary constituent of these gap junctions is connexin 26 (Cx26), a protein encoded by the gene GJB2 and found in both epithelial and connective tissue cells. It has been shown that a mutation in Cx26 accounts for 50% of patients with autosomal recessive nonsyndromic hearing loss. In the present study, we document the emergence and distribution features of Cx26 through various stages (weeks 11-31) of gestation in human, fetal cochleae. Comparative patterns of Cx26 distribution are also presented in the mature rat. The cochleae were fixed in 4% paraformaldehyde within 2 h postmortem. Immunohistochemical studies were performed using a rabbit polyclonal antibody raised against synthetic peptide and corresponding with amino acids 108-122. Specimens were mounted into paraffin sections. Results show that Cx26-like immuno reactivity is evident at a prenatal age of 11 weeks and maintains a high intensity of reactivity through 31 weeks of gestation. The appearance of this reactivity seemed to modulate in parallel with the onset of development and histological maturation as well as provide functional maintenance. In the human fetal cochlea, Cx26-like immunoreactivity distribution resembled adult patterns by fetal week 20. At the completion of morphological development by week 31, reactivity appeared to achieve an adult profile of distribution. Descriptions and discussion of Cx26 distribution patterns are presented in detail. (C) 2001 Elsevier Science B.V. All rights reserved.