Article
Impaired vascular smooth muscle cell force-generating capacity and phenotypic deregulation in Marfan Syndrome mice.
Biochimica et biophysica acta. Molecular basis of disease - 1 Jan 2020
Nolasco Patrícia, Fernandes Carolina Gonçalves, Ribeiro-Silva João Carlos, Oliveira Percillia V S, Sacrini Mariana, de Brito Isis Vasconcelos, De Bessa Tiphany Coralie, Pereira Lygia V, Tanaka Leonardo Y, Alencar Adriano, Laurindo Francisco Rafael Martins
Abstract excerpt
Mechanisms whereby fibrillin-1 mutations determine thoracic aorta aneurysms/dissections (TAAD) in Marfan Syndrome (MFS) are unclear. Most aortic aneurysms evolve from mechanosignaling deregulation, converging to impaired vascular smooth muscle cell (VSMC) force-generating capacity accompanied by synthetic phenotype switch. However, little is known on VSMC mechanoresponses in MFS pathophysiology. Here, we...
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