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进行性核上性麻痹:除神经原纤维缠结外还有广泛的神经毡丝。进行性核上性麻痹和阿尔茨海默病中皮质下神经元病变的抗原性非常相似。

Progressive supranuclear palsy: extensive neuropil threads in addition to neurofibrillary tangles. Very similar antigenicity of subcortical neuronal pathology in progressive supranuclear palsy and Alzheimer's disease.

作者信息

Probst A, Langui D, Lautenschlager C, Ulrich J, Brion J P, Anderton B H

机构信息

Department of Neuropathology, University of Basel, Switzerland.

出版信息

Acta Neuropathol. 1988;77(1):61-8. doi: 10.1007/BF00688244.

Abstract

Light microscopic immunohistochemical investigations were performed on neurofibrillary tangles (NFT) in four histologically confirmed cases of Alzheimer's disease (AD) and in five patients with a progressive supranuclear palsy (PSP). The antibody panel included antisera to the neuronal microtubule-associated protein, tau, and to isolated paired helical filaments (PHF), as well as mouse monoclonal antibodies (MAbs) to phosphorylated epitopes on high and medium molecular weight neurofilament subunits (RT97 and BF10, respectively). Paraffin sections were also impregnated with the Gallyas silver method, which specifically stains tangles and cortical neuropil threads in AD, but does not stain normal neurofilaments. All tangles in PSP and AD showed consistent immunostaining with antibodies to tau protein and isolated PHF, regardless of their localization. MAbs RT97 and BF10, however, did not stain or only weakly stained, subcortical tangles in PSP and AD, whereas most cortical NFT in AD were intensely immunostained. All tangles in PSP were as heavily impregnated with Gallyas as they were in AD. Furthermore there were extensive networks of Gallyas-positive, tau- and PHF-immunoreactive neurites in subcortical gray areas containing NFT, and bundles of positive axons in white matter tracts interconnecting subcortical nuclei of PSP. Our studies indicate a much more extensive disruption of fibrillar proteins in PSP subcortical neurons than previously reported. They furthermore indicate a very similar antigenic profile of NFT in PSP and AD, as far as subcortical neurons are concerned.

摘要

对4例经组织学确诊的阿尔茨海默病(AD)和5例进行性核上性麻痹(PSP)患者的神经原纤维缠结(NFT)进行了光镜免疫组织化学研究。抗体组包括针对神经元微管相关蛋白tau和分离的双螺旋丝(PHF)的抗血清,以及针对高分子量和中分子量神经丝亚基上磷酸化表位的小鼠单克隆抗体(分别为RT97和BF10)。石蜡切片还用Gallyas银染法处理,该方法可特异性地染出AD中的缠结和皮质神经毡丝,但不染正常神经丝。PSP和AD中的所有缠结,无论其定位如何,均与tau蛋白和分离的PHF抗体呈现一致的免疫染色。然而,单克隆抗体RT97和BF10对PSP和AD中的皮质下缠结不染色或仅弱染色,而AD中的大多数皮质NFT则呈强免疫染色。PSP中的所有缠结与AD中的缠结一样,都被Gallyas大量浸染。此外,在含有NFT的皮质下灰色区域有广泛的Gallyas阳性、tau和PHF免疫反应性神经突网络,以及在连接PSP皮质下核的白质束中有阳性轴突束。我们的研究表明,PSP皮质下神经元中纤维状蛋白的破坏比以前报道的要广泛得多。就皮质下神经元而言,它们还表明PSP和AD中NFT的抗原谱非常相似。

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