Atoh1-Lineal Neurons Are Required for Hearing and for the Survival of Neurons in the Spiral Ganglion and Brainstem Accessory Auditory Nuclei
Atoh1-Lineal Neurons Are Required for Hearing and for the Survival of Neurons in the Spiral Ganglion and Brainstem Accessory Auditory Nuclei
复制标题
DOI：
10.1523/jneurosci.2232-09.2009
复制
发表时间：
2009-09-09
影响因子：
5.3
通讯作者：
Zoghbi, Huda Y.
中科院分区：
文献类型：
--
作者：
Maricich, Stephen M.;Xia, Anping;Zoghbi, Huda Y.
Atoh 1是一种基本的螺旋-环-螺旋转录因子，对来自菱形唇的内耳毛细胞和中枢听觉系统神经元的特化是必需的。我们使用Cre-loxP系统和两条Cre-driver线（Egr 2（Cre）和Hoxb 1（Cre））从耳蜗核（CN）和副听觉核（AAN）的不同区域删除Atoh 1。成年Atoh 1-条件性基因敲除小鼠（Atoh 1（CKO））是行为性耳聋，听觉脑干诱发反应减弱，CN和AAN形态和连接中断。此外，Egr 2; Atoh 1（CKO）小鼠在生命的前3天失去耳蜗螺旋神经节神经元和AAN神经元，揭示了这些神经元发育的一个新的关键时期。这些新的小鼠模型，主要是中央耳聋照亮的CN支持外周和中枢听觉神经元的一个子集的重要性。
Atoh1 is a basic helix-loop-helix transcription factor necessary for the specification of inner ear hair cells and central auditory system neurons derived from the rhombic lip. We used the Cre-loxP system and two Cre-driver lines (Egr2(Cre) and Hoxb1(Cre)) to delete Atoh1 from different regions of the cochlear nucleus (CN) and accessory auditory nuclei (AAN). Adult Atoh1-conditional knock-out mice (Atoh1(CKO)) are behaviorally deaf, have diminished auditory brainstem evoked responses, and have disrupted CN and AAN morphology and connectivity. In addition, Egr2; Atoh1(CKO) mice lose spiral ganglion neurons in the cochlea and AAN neurons during the first 3 d of life, revealing a novel critical period in the development of these neurons. These new mouse models of predominantly central deafness illuminate the importance of the CN for support of a subset of peripheral and central auditory neurons.