The expression of α-, β-, and γ-synucleins in olfactory mucosa from patients with and without neurodegenerative diseases
The expression of α-, β-, and γ-synucleins in olfactory mucosa from patients with and without neurodegenerative diseases
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DOI：
10.1006/exnr.1999.7228
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发表时间：
1999-12-01
影响因子：
5.3
通讯作者：
Trojanowski, JQ
中科院分区：
文献类型：
--
作者：
Duda, JE;Shah, U;Trojanowski, JQ
α-、β-和γ-突触核蛋白家族在大脑中大量表达，特别是在神经元的突触前终末。尽管这些蛋白的确切功能尚不清楚，但α-突触核蛋白与鸟类鸣唱学习相关的突触可塑性以及帕金森氏病(PD)、LBS痴呆(DLB)、某些形式的阿尔茨海默病(AD)和多系统萎缩(MSA)的发病机制有关。由于嗅觉障碍是这些疾病的共同特征，而且嗅觉上皮(OE)的嗅觉感受器神经元(ON)在整个生命周期中都会再生，我们使用针对α、β和伽马突触核蛋白的抗体来检测PD、DLB、AD、MSA患者和没有神经系统疾病的对照组的嗅黏膜。虽然α-和β-突触核蛋白抗体在神经退行性疾病患者的OE中检测到异常营养不良的轴突，但在对照组的OE中也可以看到类似的病理。更重要的是，我们首次发现α-、β-和γ-突触核蛋白在嗅鞘和呼吸道上皮细胞中有不同的表达，并且α-突触核蛋白是嗅黏膜中含量最丰富的突触核蛋白，在嗅核中有显著的表达。此外，α-和γ-突触核蛋白也在OE的基底细胞中显著表达，包括OE中的Orns的祖细胞。因此，我们关于突触核蛋白在OE中表达的数据可能表明，突触核蛋白在成人OE中ONS的再生和可塑性中起作用。(C)1999年学术出版社。
A family of homologous proteins known as alpha-, beta-, and gamma-synuclein are abundantly expressed in brain, especially in the presynaptic terminal of neurons. Although the precise function of these proteins remains unknown, alpha-synuclein has been implicated in synaptic plasticity associated with avian song learning as well as in the pathogenesis of Parkinson's disease (PD), dementia with LBs (DLB), some forms of Alzheimer's disease (AD), and multiple system atrophy (MSA). Since olfactory dysfunction is a common feature of these disorders and the olfactory receptor neurons (ORNs) of the olfactory epithelium (OE) regenerate throughout the lifespan, we used antibodies specific for alpha-, beta-, and gamma-synucleins to examine the olfactory mucosa of patients with PD, DLB, AD, MSA, and controls without a neurological disorder. Although antibodies to alpha- and beta-synucleins detected abnormal dystrophic neurites in the OE of patients with neurodegenerative disorders, similar pathology was also seen in the OE of controls. More significantly, we show here for the first time that alpha-, beta-, and gamma-synucleins are differentially expressed in cells of the OE and respiratory epithelium and that alpha-synuclein is the most abundant synuclein in the olfactory mucosa, where it is prominently expressed in ORNs. Moreover, alpha- and gamma-synucleins also were prominent in the OE basal cells, which include the progenitor cells of the ORNs in the OE. Thus, our data on synuclein expression within the OE may signify that synuclein plays a role in the regeneration and plasticity of ORNs in the adult human OE. (C) 1999 Academic Press.