biostudies-literatureChen JZNCATS NIH HHSNHLBI NIH HHS2538-2550https://www.ebi.ac.uk/biostudies/studies/S-EPMC8458261biostudies-literatureUnknown41(10)Objective: A cardinal feature of Marfan syndrome is thoracic aortic aneurysm. The contribution of the renin-angiotensin system via AT1aR (Ang II [angiotensin II] receptor type 1a) to thoracic aortic aneurysm progression remains controversial because the beneficial effects of angiotensin receptor blockers have been ascribed to off-target effects. This study used genetic and pharmacological modes of attenuating angiotensin receptor and ligand, respectively, to determine their roles on thoracic aortic aneurysm in mice with fibrillin-1 haploinsufficiency (Fbn1C1041G/+).Arteriosclerosis, thrombosis, and vascular biologyDeletion of AT1a (Angiotensin II Type 1a) Receptor or Inhibition of Angiotensinogen Synthesis Attenuates Thoracic Aortopathies in Fibrillin1^C1041G/+ Mice.PMC8458261UL1 TR001998K01 HL149984F30 HL143943R01 HL133723Sheppard MBMoorleghen JJLu HSSawada HHowatt DAKukida MOhno-Urabe SFranklin MKMullick AEChen JZYe DDaugherty AKatsumata YfalseDeletion of AT1a (Angiotensin II Type 1a) Receptor or Inhibition of Angiotensinogen Synthesis Attenuates Thoracic Aortopathies in Fibrillin1^C1041G/+ Mice.Objective: A cardinal feature of Marfan syndrome is thoracic aortic aneurysm. The contribution of the renin-angiotensin system via AT1aR (Ang II [angiotensin II] receptor type 1a) to thoracic aortic aneurysm progression remains controversial because the beneficial effects of angiotensin receptor blockers have been ascribed to off-target effects. This study used genetic and pharmacological modes of attenuating angiotensin receptor and ligand, respectively, to determine their roles on thoracic aortic aneurysm in mice with fibrillin-1 haploinsufficiency (Fbn1C1041G/+).2021-01-01T00:00:00Z2021 Oct2026-03-31T10:53:02.414Z2025-02-19T01:22:10.363ZS-EPMC84582613440763410.1161/ATVBAHA.121.31571510.1161/atvbaha.121.315715