Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas, USA.
Nat Genet. 2011 Sep 11;43(10):996-1000. doi: 10.1038/ng.934.
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.
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。