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全基因组关联研究鉴定出 15q21.1 上跨越 FBN1 的胸主动脉瘤和主动脉夹层易感性位点。

Genome-wide association study identifies a susceptibility locus for thoracic aortic aneurysms and aortic dissections spanning FBN1 at 15q21.1.

机构信息

Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas, USA.

出版信息

Nat Genet. 2011 Sep 11;43(10):996-1000. doi: 10.1038/ng.934.

Abstract

Although thoracic aortic aneurysms and dissections (TAAD) can be inherited as a single-gene disorder, the genetic predisposition in the majority of affected people is poorly understood. In a multistage genome-wide association study (GWAS), we compared 765 individuals who had sporadic TAAD (STAAD) with 874 controls and identified common SNPs at a 15q21.1 locus that were associated with STAAD, with odds ratios of 1.6-1.8 that achieved genome-wide significance. We followed up 107 SNPs associated with STAAD with P < 1 × 10(-5) in the region, in two separate STAAD cohorts. The associated SNPs fall into a large region of linkage disequilibrium encompassing FBN1, which encodes fibrillin-1. FBN1 mutations cause Marfan syndrome, whose major cardiovascular complication is TAAD. This study shows that common genetic variants at 15q21.1 that probably act via FBN1 are associated with STAAD, suggesting a common pathogenesis of aortic disease in Marfan syndrome and STAAD.

摘要

虽然胸主动脉瘤和夹层(TAAD)可以作为单基因疾病遗传,但大多数受影响者的遗传易感性仍不清楚。在一项多阶段全基因组关联研究(GWAS)中,我们比较了 765 例散发性 TAAD(STAAD)患者与 874 例对照者,在 15q21.1 位点发现了与 STAAD 相关的常见 SNP,其比值比为 1.6-1.8,达到了全基因组显著水平。我们对该区域中与 STAAD 相关的 107 个 SNP(P < 1×10(-5))进行了随访,在两个独立的 STAAD 队列中进行了随访。相关 SNP 落入包含编码原纤维蛋白-1 的 FBN1 的大片段连锁不平衡区域。FBN1 突变导致马凡综合征,其主要心血管并发症是 TAAD。这项研究表明,15q21.1 上的常见遗传变异可能通过 FBN1 与 STAAD 相关,提示马凡综合征和 STAAD 的主动脉疾病具有共同的发病机制。

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