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家族性和早发性阿尔茨海默病的基因检测:塞尔维亚队列中的突变谱。

Genetic testing in familial and young-onset Alzheimer's disease: mutation spectrum in a Serbian cohort.

机构信息

Institute of Neurology, Clinical Center of Serbia, Belgrade, Serbia.

出版信息

Neurobiol Aging. 2012 Jul;33(7):1481.e7-12. doi: 10.1016/j.neurobiolaging.2011.12.007. Epub 2012 Jan 4.

Abstract

Alzheimer's disease (AD) is the most common form of dementia. To date, more than 200 mutations in three genes have been identified as cause of early-onset autosomal dominant inherited AD. The aim of this study was to characterize the mutation spectrum and describe genotype-phenotype correlations in Serbian patients with positive family history of AD or/and early-onset AD. We performed a genetic screening for mutations in the coding regions of Presenilins 1 and 2 (PSEN1 and PSEN2), as well as exons 16 and 17 of the Amyloid Precursor Protein gene (APP) in a total of 47 patients from Serbia with a clinical diagnosis of familial and/or early-onset AD (mean age at onset of 60.3 years; range 32-77). We found one novel mutation in PSEN1, one novel variant in PSEN2, and three previously described variants, one in each of the analyzed genes. Interestingly, we identified one patient harboring two heterozygous mutations: one in APP (p.L723P) and one in PSEN1 (p.R108Q).

摘要

阿尔茨海默病(AD)是最常见的痴呆症形式。迄今为止,已经在三个基因中的 200 多个突变中发现了导致早发性常染色体显性遗传性 AD 的原因。本研究的目的是描述塞尔维亚具有 AD 阳性家族史或/和早发性 AD 的患者的突变谱,并描述基因型-表型相关性。我们对来自塞尔维亚的共 47 名具有家族性和/或早发性 AD 临床诊断的患者进行了编码区 Presenilins 1 和 2(PSEN1 和 PSEN2)以及淀粉样前体蛋白基因(APP)外显子 16 和 17 的突变遗传筛查(发病年龄的平均值为 60.3 岁;范围 32-77)。我们发现 PSEN1 中有一个新的突变,PSEN2 中有一个新的变体,以及三个先前描述的变体,每个分析的基因中都有一个。有趣的是,我们鉴定了一名携带两个杂合突变的患者:一个在 APP(p.L723P)中,一个在 PSEN1(p.R108Q)中。

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