Effects of the inhibition of the angiotensin converting enzyme in osteogenic diffe...
Evaluation of Fibrillin-1's role in arterial thrombogenesis.
Generation of human endothelial cells derived from hiPSC lineages with FBN1 mutations
Grant number: | 10/19916-7 |
Support Opportunities: | Regular Research Grants |
Field of knowledge: | Biological Sciences - Biochemistry - Molecular Biology |
Principal Investigator: | Claudio Chrysostomo Werneck |
Grantee: | Claudio Chrysostomo Werneck |
Host Institution: | Instituto de Biologia (IB). Universidade Estadual de Campinas (UNICAMP). Campinas , SP, Brazil |
Associated researchers: | Cristina Pontes Vicente ; Guilherme Gambogi Braga |
Abstract
Fibrillin-1 is an important microfibril network component. Microfibrils are present in elastic fiber responsible for resilience and elastic properties from structures like lungs, skin and large vessels. Mutations in Fibrillin-1 gene are associated with Marfan's Syndrome. Marfan's Syndrome patients shown many different clinical manifestations in lungs, cardiovascular tract and eyes tissues. Marfan's models have been created to get better insights about this disease development. Recent studies from mice models have suggested an important role to unbalanced TGF beta activity promoting almost whole alterations found in those mice which might be rescued by losartan treatment. Our main goal is evaluate elastic fiber formation in cell culture obtained from fibrillin-1 defficient mice and verify TGF-beta expression levels as well as losartan's treatment effect on those cells. (AU)
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