Ang II type 1a receptor in Marfan syndrome

Using Cdh5-Cre and Sm22-Cre transgenes to characterize the impact of disruption of the angiotensin II type 1a receptor (AT1ar) in vascular endothelial and smooth muscle cells, respectively, of wild type (WT) mice compared to fibrillin-1 hypomorphic mice (Fbn1mgR/mgR mice) that replicate early onset progressively severe Marfan syndrome (MFS) with dissecting thoracic aortic aneurysm (TAA). Comparing biological processes identified from transcriptomic analyses of aortic tissue harvested from either WT (at1Rfl) mice or mice with a knockout of AT1ar in either endothelial cells (at1Rcdh5) or smooth muscle cells (at1Rsm22).本研究借助Cdh5-Cre与Sm22-Cre转基因工具，分别在野生型（WT）小鼠的血管内皮细胞和平滑肌细胞中敲除血管紧张素II 1a型受体（angiotensin II type 1a receptor, AT1ar）以表征其生物学效应，并与可模拟早发性进行性重症马凡综合征（Marfan syndrome, MFS）伴夹层胸主动脉瘤（dissecting thoracic aortic aneurysm, TAA）的原纤维蛋白-1低突变小鼠（Fbn1mgR/mgR小鼠）进行对照分析。本研究还对分别取自野生型（at1Rfl）小鼠、内皮细胞AT1ar特异性敲除（at1Rcdh5）小鼠或平滑肌细胞AT1ar特异性敲除（at1Rsm22）小鼠的主动脉组织开展转录组学分析，对比各组样本所鉴定得到的生物学过程。