Department of Neurology, and Department of Psychiatry, Semel Institute, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California 90095, Imaging Genetics Center, and Departments of Neurology, and Psychiatry, Radiology, Engineering, Pediatrics, and Ophthalmology, Keck School of Medicine, University of Southern California, Los Angeles, California 90033.
J Neurosci. 2014 May 7;34(19):6537-45. doi: 10.1523/JNEUROSCI.5236-13.2014.
The C allele at the rs11136000 locus in the clusterin (CLU) gene is the third strongest known genetic risk factor for late-onset Alzheimer's disease (LOAD). A recent genome-wide association study of LOAD found the strongest evidence of association with CLU at rs1532278, in high linkage disequilibrium with rs11136000. Brain structure and function are related to the CLU risk alleles, not just in LOAD patients but also in healthy young adults. We tracked the volume of the lateral ventricles across baseline, 1-year, and 2-year follow-up scans in a large sample of elderly human participants (N = 736 at baseline), from the Alzheimer's Disease Neuroimaging Initiative, to determine whether these CLU risk variants predicted longitudinal ventricular expansion. The rs11136000 major C allele-previously linked with reduced CLU expression and with increased risk for dementia-predicted faster expansion, independently of dementia status or ApoE genotype. Further analyses revealed that the CLU and ApoE risk variants had combined effects on both volumetric expansion and lateral ventricle surface morphology. The rs1532278 locus strongly resembles a regulatory element. Its association with ventricular expansion was slightly stronger than that of rs11136000 in our analyses, suggesting that it may be closer to a functional variant. Clusterin affects inflammation, immune responses, and amyloid clearance, which in turn may result in neurodegeneration. Pharmaceutical agents such as valproate, which counteract the effects of genetically determined reduced clusterin expression, may help to achieve neuroprotection and contribute to the prevention of dementia, especially in carriers of these CLU risk variants.
CLU 基因 rs11136000 位点的 C 等位基因是已知的第三个导致晚发性阿尔茨海默病(LOAD)的最强遗传风险因素。最近一项针对 LOAD 的全基因组关联研究发现,CLU 基因 rs1532278 与 rs11136000 高度连锁不平衡,与 LOAD 关联最强。CLU 风险等位基因与大脑结构和功能有关,不仅在 LOAD 患者中,而且在健康的年轻成年人中也是如此。我们从阿尔茨海默病神经影像学倡议(Alzheimer's Disease Neuroimaging Initiative)中追踪了大量老年人类参与者(基线时为 736 名)的侧脑室体积在基线、1 年和 2 年随访扫描中的变化,以确定这些 CLU 风险变体是否预测纵向脑室扩张。rs11136000 主要 C 等位基因与 CLU 表达减少和痴呆风险增加有关,与痴呆状态或 ApoE 基因型无关,可预测更快的扩张。进一步的分析表明,CLU 和 ApoE 风险变体对体积扩张和侧脑室表面形态都有联合作用。rs1532278 位点与调节元件非常相似。在我们的分析中,它与脑室扩张的关联比 rs11136000 略强,这表明它可能更接近功能性变体。Clusterin 影响炎症、免疫反应和淀粉样蛋白清除,这反过来可能导致神经退行性变。丙戊酸等药物可以抵消遗传决定的 Clusterin 表达减少的影响,可能有助于实现神经保护,并有助于预防痴呆症,特别是在这些 CLU 风险变体的携带者中。