Canal cristae growth and fiber extension to the outer hair cells of the mouse ear require Prox1 activity.
Canal cristae growth and fiber extension to the outer hair cells of the mouse ear require Prox1 activity.
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DOI：
10.1371/journal.pone.0009377
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发表时间：
2010-02-23
期刊：
影响因子：
3.7
通讯作者：
Jahan I
中科院分区：
文献类型：
--
作者：
Fritzsch B;Dillard M;Lavado A;Harvey NL;Jahan I
同源框基因 Prox1 是晶状体、视网膜、胰腺、肝脏和淋巴管系统发育所必需的，在内耳支持细胞和神经元中表达。我们利用 Tg(Pax2-Cre) 和 Tg(Nes-Cre) 现有的标准和条件 Prox1 突变小鼠品系研究了 Prox1 在小鼠耳发育中的作用。在 E18.5 Prox1Flox/Flox 中发现耳管嵴尺寸严重缩小，但其他前庭器官或耳蜗并未严重缩小； Tg(Pax2-Cre)突变耳。在这些突变胚胎中，毛细胞发生分化；然而，它们在耳蜗中的分布模式略有混乱，其中 II 型神经纤维沿着表达 Prox1 的支持细胞向外毛细胞的生长受到严重破坏。就 Nestin-Cre 而言，我们发现新生的 Prox1Flox/Flox；尽管柯蒂氏器的细胞分化明显正常，但 Tg(Nestin-Cre) 仅表现出外毛细胞的神经支配紊乱，这表明 Prox1 在神经元中具有细胞自主功能。这些结果确定了 Prox1 在内耳发育过程中的双重作用；耳蜗嵴的生长和 II 型纤维沿着耳蜗支持细胞的纤维引导。
The homeobox gene Prox1 is required for lens, retina, pancreas, liver, and lymphatic vasculature development and is expressed in inner ear supporting cells and neurons. We have investigated the role of Prox1 in the developing mouse ear taking advantage of available standard and conditional Prox1 mutant mouse strains using Tg(Pax2-Cre) and Tg(Nes-Cre). A severe reduction in the size of the canal cristae but not of other vestibular organs or the cochlea was identified in the E18.5 Prox1Flox/Flox; Tg(Pax2-Cre) mutant ear. In these mutant embryos, hair cell differentiated; however, their distribution pattern was slightly disorganized in the cochlea where the growth of type II nerve fibers to outer hair cells along Prox1 expressing supporting cells was severely disrupted. In the case of Nestin-Cre, we found that newborn Prox1Flox/Flox; Tg(Nestin-Cre) exhibit only a disorganized innervation of outer hair cells despite apparently normal cellular differentiation of the organ of Corti, suggesting a cell-autonomous function of Prox1 in neurons. These results identify a dual role of Prox1 during inner ear development; growth of the canal cristae and fiber guidance of Type II fibers along supporting cells in the cochlea.
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影响因子：
64.8
作者：
BenArie, N;Bellen, HJ;Zoghbi, HY
通讯作者：
Zoghbi, HY
DOI：
10.1073/pnas.0808175105
发表时间：
2008-11-25
影响因子：
11.1
作者：
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作者：
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通讯作者：
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影响因子：
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作者：
Bermingham, NA;Hassan, BA;Zoghbi, HY
通讯作者：
Zoghbi, HY