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成年小鼠的嗅觉感觉神经元中表达细胞朊蛋白,但不会影响嗅觉转导途径的早期事件。

The cellular prion protein is expressed in olfactory sensory neurons of adult mice but does not affect the early events of the olfactory transduction pathway.

机构信息

SISSA, International School for Advanced Studies and Italian Institute of Technology, SISSA Unit, Trieste, Italy.

出版信息

Chem Senses. 2011 Nov;36(9):791-7. doi: 10.1093/chemse/bjr054. Epub 2011 Jun 16.

Abstract

A conformational conversion of the cellular prion protein (PrP(C)) is now recognized as the causal event of fatal neurodegenerative disorders, known as prion diseases. In spite of long-lasting efforts, however, the physiological role of PrP(C) remains unclear. It has been reported that PrP(C) is expressed in various areas of the olfactory system, including the olfactory epithelium, but its precise localization in olfactory sensory neurons (OSNs) is still debated. Here, using immunohistochemistry tools, we have reinvestigated the expression and localization of PrP(C) in the olfactory epithelium of adult congenic mice expressing different PrP(C) amounts, that is, wild-type, PrP-knockout, and transgenic PrP(C)-overexpressing animals. We found that PrP(C) was expressed in OSNs, in which, however, it was unevenly distributed, being detectable at low levels in cell bodies, dendrites and apical layer, and more abundantly in axons. We also studied the involvement of PrP(C) in the response of the olfactory epithelium to odorants, by comparing the electro-olfactograms of the 3 mouse lines subjected to different stimulation protocols. We found no significant difference between the 3 PrP genotypes, supporting previous reports that exclude a direct action of PrP(C) in the early signal transduction activity of the olfactory epithelium.

摘要

细胞朊蛋白(PrP(C))的构象转换现在被认为是导致致命神经退行性疾病(称为朊病毒病)的原因。然而,尽管经过长期努力,PrP(C)的生理作用仍不清楚。据报道,PrP(C)在嗅觉系统的各个区域表达,包括嗅上皮,但它在嗅觉感觉神经元(OSN)中的精确定位仍存在争议。在这里,我们使用免疫组织化学工具,重新研究了在表达不同 PrP(C)量的同源小鼠的嗅上皮中 PrP(C)的表达和定位,即野生型、PrP 敲除和转基因 PrP(C)过表达动物。我们发现 PrP(C)在 OSN 中表达,但分布不均匀,在细胞体、树突和顶层层中检测到低水平,在轴突中更丰富。我们还通过比较 3 种小鼠品系在不同刺激方案下的电嗅图,研究了 PrP(C)在嗅上皮对气味剂的反应中的参与。我们没有发现 3 种 PrP 基因型之间存在显著差异,这支持了先前的报告,即排除了 PrP(C)在嗅上皮早期信号转导活性中的直接作用。

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