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神经退行性疾病中的多蛋白沉积:我们在纳瓦拉脑组织库的经验。

Multiprotein deposits in neurodegenerative disorders: our experience in the tissue brain bank of Navarra.

机构信息

Brain Bank of Navarre, Biomedical Research Center, Pamplona, Spain.

出版信息

Anat Rec (Hoboken). 2011 Jul;294(7):1191-7. doi: 10.1002/ar.21413. Epub 2011 May 26.

Abstract

The prevalence of neurodegenerative disorders increases dramatically with advancing age. Although in recent decades the study of many neurodegenerative disorders has evolved greatly, the concept of neurodegeneration still remains elusive. Although neurodegenerative disorders are classified according to the major components of protein deposits, coexpression of several abnormal proteins in the brain tissue is more common than that was previously thought. The aim of this report is to describe the type of protein deposits found in brains with neuropathological diagnosis of neurodegenerative disease. The report shows the experience obtained in the Brain Bank of Navarra (Spain). The target population for this retrospective descriptive study comprised 178 brains autopsied in the "Hospital of Navarra" in Pamplona between 1994 and 2004 and 201 brains donated to the Brain Bank of Pamplona between 2004 and 2009. The diagnosis of the 201 brains from the Brain Bank was 62 (30.8%) Alzheimer's disease (AD), 43 (21.3%) multiprotein deposit, 31 (15.4%) α-synucleinopathies, 31 (15.4%) frontotemporal lobar degeneration (FTLD), 17 (8.4%) tauopathies, 9 (4.4%) prion disease, 6 (2.9%) vascular dementia (VD), and 2 (0.9%) Huntington's disease. Among the 43 cases with multiprotein deposits, we found 35 brains with deposits of 3 proteins (tau, β-amyloid, and α-synuclein). In these two series of brains, the high incidence of deposition of multiple proteins in neurodegenerative disorders is shown. Our results are in agreement with previous findings showing that tau, β-amyloid, and α-synuclein are the proteins most frequently deposited together.

摘要

神经退行性疾病的患病率随着年龄的增长而急剧增加。尽管在最近几十年中,许多神经退行性疾病的研究有了很大的发展,但神经退行性的概念仍然难以捉摸。尽管神经退行性疾病是根据蛋白质沉积物的主要成分进行分类的,但脑组织中几种异常蛋白质的共表达比以前认为的更为常见。本报告的目的是描述具有神经退行性疾病病理诊断的脑组织中发现的蛋白质沉积物类型。该报告展示了在西班牙纳瓦拉脑库(Brain Bank of Navarra)获得的经验。这项回顾性描述性研究的目标人群包括 1994 年至 2004 年在潘普洛纳的“纳瓦拉医院”进行尸检的 178 例大脑,以及 2004 年至 2009 年捐赠给潘普洛纳脑库的 201 例大脑。脑库中 201 例大脑的诊断结果为 62 例(30.8%)阿尔茨海默病(AD)、43 例(21.3%)多种蛋白沉积、31 例(15.4%)α-突触核蛋白病、31 例(15.4%)额颞叶变性(FTLD)、17 例(8.4%)tau 病、9 例(4.4%)朊病毒病、6 例(2.9%)血管性痴呆(VD)和 2 例(0.9%)亨廷顿病。在 43 例多种蛋白沉积病例中,我们发现 35 例大脑中有 3 种蛋白(tau、β-淀粉样蛋白和 α-突触核蛋白)沉积。在这两组大脑中,显示出神经退行性疾病中多种蛋白质沉积的高发生率。我们的结果与先前的发现一致,表明 tau、β-淀粉样蛋白和 α-突触核蛋白是最常共同沉积的蛋白质。

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