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家族性和散发性阿尔茨海默病中β-淀粉样蛋白(Aβ)沉积的空间模式。

Spatial patterns of β-amyloid (Aβ) deposits in familial and sporadic Alzheimer's disease.

机构信息

Vision Sciences, Aston University, Birmingham B4 7ET, UK.

出版信息

Folia Neuropathol. 2011;49(3):153-61.

Abstract

The spatial patterns of the diffuse, primitive, and classic β -amyloid (Aβ) deposits were compared in cortical regions in early-onset familial Alzheimer's disease (EO-FAD) linked to mutations of the amyloid precursor protein APP) or presenilin 1 (PSEN1) genes, late-onset familial AD (LO-FAD), and sporadic AD (SAD). The objective was to determine whether genetic factors influenced the spatial patterns of the A β deposits. A β deposits were distributed either in clusters which were regularly distributed parallel to the pia mater or in larger, non-regularly distributed clusters. There were no significant differences in spatial pattern of the diffuse deposits between patient groups but mean cluster size of the diffuse deposits was larger in FAD compared with SAD. Primitive A β deposits were more frequently distributed in regular clusters and less frequently distributed in large clusters in FAD compared with SAD. Classic A β deposits were more frequently distributed in regularly spaced clusters and less frequently distributed in large clusters in LO-FAD compared with EO-FAD. There were no significant differences in the spatial patterns or cluster sizes of A β deposits in cases classified according to apolipoprotein E (APOE) genotype. These results suggest (1) greater deposition of A β in the form of clusters of diffuse deposits in FAD, (2) a greater proportion of diffuse deposits may be converted to primitive deposits in SAD, (3) classic deposits are more widely distributed in EO-FAD, and (4) the presence of APOE allele ε4 has little effect on the spatial patterns of A β deposits.

摘要

皮质区弥漫性、原始性和经典β-淀粉样蛋白(Aβ)沉积的空间模式在早发性家族性阿尔茨海默病(EO-FAD)、晚发性家族性 AD(LO-FAD)和散发性 AD(SAD)患者中进行了比较,这些患者与淀粉样前体蛋白(APP)或早老素 1(PSEN1)基因突变有关。目的是确定遗传因素是否影响 Aβ沉积的空间模式。Aβ沉积要么呈平行于软脑膜规则分布的簇状分布,要么呈较大的、不规则分布的簇状分布。各组间弥漫性沉积的空间模式无显著差异,但 FAD 中弥漫性沉积的平均簇大小大于 SAD。与 SAD 相比,原始 Aβ沉积在 FAD 中更常呈规则簇状分布,而较少呈大簇状分布。与 EO-FAD 相比,经典 Aβ沉积在 LO-FAD 中更常呈规则间隔的簇状分布,而较少呈大簇状分布。根据载脂蛋白 E(APOE)基因型分类的病例中,Aβ沉积的空间模式或簇大小无显著差异。这些结果表明:(1)FAD 中弥漫性沉积簇中 Aβ沉积量增加;(2)SAD 中可能有更多的弥漫性沉积转化为原始沉积;(3)经典沉积在 EO-FAD 中分布更广;(4)APOE 等位基因 ε4 的存在对 Aβ沉积的空间模式影响不大。

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