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唐氏综合征患者:细胞外淀粉样前体沉积先于神经突退变和老年斑出现。

Down patients: extracellular preamyloid deposits precede neuritic degeneration and senile plaques.

作者信息

Giaccone G, Tagliavini F, Linoli G, Bouras C, Frigerio L, Frangione B, Bugiani O

机构信息

Istituto neurologico Carlo Besta, Milan, Italy.

出版信息

Neurosci Lett. 1989 Feb 13;97(1-2):232-8. doi: 10.1016/0304-3940(89)90169-9.

DOI:10.1016/0304-3940(89)90169-9
PMID:2521927
Abstract

Using anti-SP28 (a polyclonal antibody to a 28 residue synthetic peptide homologous to the NH2-terminal region of the Alzheimer amyloid beta-protein) to investigate the cerebral cortex of 6 Down patients aged 6-55 y, we found that, besides senile plaques and congophilic vessels, extracellular deposits unrelated to degenerating neurites, tangle-bearing neurons or congophilic vessels were labelled. These deposits were similar to the extracellular deposits previously observed in the cerebral cortex of Alzheimer patients and non-demented individuals. The material accumulated in the deposits did not react with Congo red, thioflavine S or, on some occasions, silver salts and therefore might have been constituted by beta-protein precursors lacking the molecular conformation of amyloid fibrils. Age-related analysis of the cortical lesions in Down patients suggested that such extracellular deposits precede degenerating neurites and evolve into senile plaques.

摘要

使用抗SP28(一种针对与阿尔茨海默病淀粉样β蛋白NH2末端区域同源的28个残基合成肽的多克隆抗体)对6名年龄在6至55岁的唐氏综合征患者的大脑皮层进行研究,我们发现,除了老年斑和嗜刚果红血管外,与神经突退变、缠结神经元或嗜刚果红血管无关的细胞外沉积物也被标记。这些沉积物与先前在阿尔茨海默病患者和非痴呆个体大脑皮层中观察到的细胞外沉积物相似。沉积物中积累的物质不与刚果红、硫黄素S反应,在某些情况下也不与银盐反应,因此可能由缺乏淀粉样纤维分子构象的β蛋白前体构成。对唐氏综合征患者皮层病变的年龄相关性分析表明,此类细胞外沉积物先于神经突退变,并演变为老年斑。

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Down patients: extracellular preamyloid deposits precede neuritic degeneration and senile plaques.唐氏综合征患者:细胞外淀粉样前体沉积先于神经突退变和老年斑出现。
Neurosci Lett. 1989 Feb 13;97(1-2):232-8. doi: 10.1016/0304-3940(89)90169-9.
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