Claudin7b is required for the formation and function of inner ear in zebrafish
Claudin7b is required for the formation and function of inner ear in zebrafish
复制标题
斑马鱼内耳的形成和功能需要 Claudin7b
DOI：
10.1002/jcp.26162
复制
发表时间：
2018-04-01
影响因子：
5.6
通讯作者：
Cui, Zongbin
中科院分区：
文献类型：
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作者：
Li, Xiaohui;Song, Guili;Cui, Zongbin
斑马鱼已成为研究内耳发育和功能的极好模型。我们在这里报告的斑马鱼线，其中claudin 7b（cldn7b）基因座被中断的Tol2转座子在其第一个内含子。纯合子突变体的耳囊增大，耳石变小或没有耳石，半规管缓慢形成，对声音刺激不敏感。这些异常表型和内耳听力损失可以通过将cldn7b-mRNA注射到单细胞期纯合突变胚胎中来大部分挽救。从机制上讲，cldn7b-缺陷中断了内耳耳上皮细胞中顶端连接复合体（AJC）的形成和内淋巴的离子稳态，然后导致耳石与椭圆囊和球囊中正常发育的毛细胞之间的适当接触丧失或异常的机械感觉转导。因此，Cldn7b通过其在斑马鱼胚胎发育期间保持耳上皮初始完整性的独特作用，对内耳的形成和适当功能至关重要。
Zebrafish has become an excellent model for studying the development and function of inner ear. We report here a zebrafish line in which claudin 7b (cldn7b) locus is interrupted by a Tol2 transposon at its first intron. The homozygous mutants have enlarged otocysts, smaller or no otoliths, slowly formed semicircular canals, and insensitiveness to sound stimulation. These abnormal phenotypes and hearing loss of inner ear could be mostly rescued by injection of cldn7b‐mRNA into one‐cell stage homozygous mutant embryos. Mechanistically, cldn7b‐deficiency interrupted the formation of apical junction complexes (AJCs) in otic epithelial cells of inner ear and the ion‐homeostasis of endolymph, which then led to the loss of proper contact between otoliths and normally developed hair cells in utricle and saccule or aberrant mechanosensory transduction. Thus, Cldn7b is essential for the formation and proper function of inner ear through its unique role in keeping an initial integrity of otic epithelia during zebrafish embryogenesis.