保持发育自发活动使聋小鼠的听觉系统能够早期成熟
Calvin J Kersbergen1, Travis A Babola1,2, Patrick O Kanold2
1The Solomon H. Snyder Department of Neuroscience, Johns Hopkins University, Baltimore, Maryland, United States of America.
PLoS biology
|June 27, 2023
概括
与连结素26 (Gjb2) 突变相关的先天性聋可能不会完全破坏早期听觉电路的发育. 耳中的自发活动被保留,这表明在听力发作之前可能需要进行治疗干预.
科学领域:
- 神经科学是一个神经科学.
- 发展生物学 发展生物学
- 遗传学 是一个遗传学.
背景情况:
- 在听力之前,内在产生的神经活动对听觉系统的成熟至关重要.
- 这种活动源于Corti器官中的支持细胞,这些细胞通过连接素26 (Gjb2) 间隙结相互连接.
- Gjb2突变是先天性聋的主要原因,但它们对早期听觉电路发育的影响尚不清楚.
研究的目的:
- 调查Gjb2变种是否会扰乱大脑中自发活动和听觉电路的发展.
- 为了检查Gjb2在支持细胞合和神经活动在听力发作之前的作用.
主要方法:
- 使用了一种新的小鼠模型来治疗Gjb2介导的先天性聋.
- 评估了耳支细胞中的细胞间合和自发活动.
- 在中央听觉神经元中记录神经活动.
- 检查了发细胞完整性和听觉神经元兴奋度听力发病后.
主要成果:
- 缺少Gjb2的耳支细胞保留了细胞间合和自发活动,在听力发作之前只有轻微的缺陷.
- 这种活动协调了内毛细胞的激活,并导致中央听觉神经元中的同步爆发.
- 听觉回路成熟得恰当,毛细胞完好,神经元在听觉开始时被大声声音激活.
- 渐进的毛细胞退化和增加的神经元刺激性仅在听力开始和自发活动停止后才发生.
结论:
- 在Gjb2缺乏的小鼠中,尽管有耳变化,听觉回路的早期成熟和精细化仍保留着.
- 在Gjb2缺失的情况下保存自发的神经活动为治疗干预提供了潜在的窗口.
- 这一发现表明,由Gjb2突变引起的先天性聋可能不会阻碍关键的早期听觉通路的发展.
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