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脆性X智力低下蛋白在双耳脑干神经元中的强烈且特异性树突定位:短吻鳄、鸡、沙鼠和人类的比较研究

Intense and specialized dendritic localization of the fragile X mental retardation protein in binaural brainstem neurons: a comparative study in the alligator, chicken, gerbil, and human.

作者信息

Wang Yuan, Sakano Hitomi, Beebe Karisa, Brown Maile R, de Laat Rian, Bothwell Mark, Kulesza Randy J, Rubel Edwin W

机构信息

Virginia Merrill Bloedel Hearing Research Center, Department of Otolaryngology-Head and Neck Surgery, University of Washington School of Medicine, Seattle, WA, 98195-7923.

出版信息

J Comp Neurol. 2014 Jun 15;522(9):2107-28. doi: 10.1002/cne.23520.

Abstract

Neuronal dendrites are structurally and functionally dynamic in response to changes in afferent activity. The fragile X mental retardation protein (FMRP) is an mRNA binding protein that regulates activity-dependent protein synthesis and morphological dynamics of dendrites. Loss and abnormal expression of FMRP occur in fragile X syndrome (FXS) and some forms of autism spectrum disorders. To provide further understanding of how FMRP signaling regulates dendritic dynamics, we examined dendritic expression and localization of FMRP in the reptilian and avian nucleus laminaris (NL) and its mammalian analogue, the medial superior olive (MSO), in rodents and humans. NL/MSO neurons are specialized for temporal processing of low-frequency sounds for binaural hearing, which is impaired in FXS. Protein BLAST analyses first demonstrate that the FMRP amino acid sequences in the alligator and chicken are highly similar to human FMRP with identical mRNA-binding and phosphorylation sites, suggesting that FMRP functions similarly across vertebrates. Immunocytochemistry further reveals that NL/MSO neurons have very high levels of dendritic FMRP in low-frequency hearing vertebrates including alligator, chicken, gerbil, and human. Remarkably, dendritic FMRP in NL/MSO neurons often accumulates at branch points and enlarged distal tips, loci known to be critical for branch-specific dendritic arbor dynamics. These observations support an important role for FMRP in regulating dendritic properties of binaural neurons that are essential for low-frequency sound localization and auditory scene segregation, and support the relevance of studying this regulation in nonhuman vertebrates that use low frequencies in order to further understand human auditory processing disorders.

摘要

神经元树突在传入活动发生变化时,其结构和功能具有动态性。脆性X智力低下蛋白(FMRP)是一种mRNA结合蛋白,可调节依赖活动的蛋白质合成以及树突的形态动力学。FMRP的缺失和异常表达出现在脆性X综合征(FXS)和某些形式的自闭症谱系障碍中。为了进一步了解FMRP信号如何调节树突动力学,我们研究了FMRP在爬行动物和鸟类的层状核(NL)及其在啮齿动物和人类中的哺乳动物类似物内侧上橄榄核(MSO)中的树突表达和定位。NL/MSO神经元专门用于对低频声音进行时间处理以实现双耳听觉,而在FXS中这种功能会受损。蛋白质BLAST分析首先表明,短吻鳄和鸡的FMRP氨基酸序列与人类FMRP高度相似,具有相同的mRNA结合和磷酸化位点,这表明FMRP在整个脊椎动物中功能相似。免疫细胞化学进一步揭示,在包括短吻鳄、鸡、沙鼠和人类在内的低频听力脊椎动物中,NL/MSO神经元的树突FMRP水平非常高。值得注意的是,NL/MSO神经元中的树突FMRP通常聚集在分支点和扩大的远端末梢,这些位点已知对分支特异性树突分支动力学至关重要。这些观察结果支持了FMRP在调节双耳神经元树突特性方面的重要作用,而这些特性对于低频声音定位和听觉场景分离至关重要,并且支持在使用低频的非人类脊椎动物中研究这种调节的相关性,以便进一步了解人类听觉处理障碍。

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