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Biomolecules, Volume 12, Issue 10
October 2022 - 216 articles
Cover Story: Fibrillin-1 is an extracellular matrix protein that provides critical functions in connective tissues. Mutations of this protein give rise to Marfan syndrome, a genetic disease characterized by severe cardiovascular, ocular, and skeletal symptoms. Typically, fibrillin-1 mutations cause the progressive enlargement of the ascending aorta, leading to aneurysms and dissections. Marfan patients also suffer from retinal detachment. However, if and how fibrillin-1 deficiency affects the microvasculature have never been investigated. We show that fibrillin-1 plays a critical role in the homeostasis of retinal arterioles. Fibrillin-1 deficiency impairs basement membrane and smooth muscle cell coverage, resulting in arteriolar leakage and enlargement. Losartan treatment, which reduces aortic root dilation in mouse models of Marfan syndrome, rescues the arteriolar defects. View this paper
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Biomolecules - ISSN 2218-273X