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JCI Insight:TRIOBP-5能够塑造静纤毛根并强化支持细胞从而实现听力功能

2019-07-07 AlexYang MedSci原创

TRIOBP能够通过形成异常致密的F-肌动蛋白来重塑细胞骨架,并且在人类癌症、精神分裂症和耳聋中具有作用。人类和小鼠TRIOBP-4和TRIOBP-5的突变与永久性听力损失有关,原因是静纤毛小根发育不全导致了内耳机械感觉听毛细胞的退化。然而,静纤毛根形成具体是通过TRIOBP不同亚型怎样调控的仍旧未知。最近,有研究人员使用小鼠模型报道了TRIOBP-5对加厚静纤毛根F肌动蛋白束和建立成熟的尺寸是必

TRIOBP能够通过形成异常致密的F-肌动蛋白来重塑细胞骨架,并且在人类癌症、精神分裂症和耳聋中具有作用。人类和小鼠TRIOBP-4和TRIOBP-5的突变与永久性听力损失有关,原因是静纤毛小根发育不全导致了内耳机械感觉听毛细胞的退化。然而,静纤毛根形成具体是通过TRIOBP不同亚型怎样调控的仍旧未知。

最近,有研究人员使用小鼠模型报道了TRIOBP-5对加厚静纤毛根F肌动蛋白束和建立成熟的尺寸是必要的,且对听觉感知上皮支持细胞的加固是必需的。另外,该蛋白亚型的卷曲螺旋结构对静纤毛根的加固和维持是需要的。TRIOBP-5功能的丧失能够导致异常的静纤毛根,其厚度小但宽度和长度增加,并能够引起渐进性耳聋,与人类的表型一致。

最后,研究人员指出,他们的研究扩展了人们对TRIOBP亚型对长久听力维持的必要性的认识,也表明了其它TRIOBP亚型在其中可能的作用。

原始出处:

Katsuno T, Belyantseva IA, Cartagena-Rivera AX et al. TRIOBP-5 sculpts stereocilia rootlets and stiffens supporting cells enabling hearing. JCI Insight. 20 Jun 2019

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    2019-12-14 caobinglei8079
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    2019-07-09 ysjykql

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