Francesco Ramirez, Ph.D.
Department of Pharmacology& Systems Therapeutics
Mount Sinai School of Medicine, New York, NY
A major focus of our research program is the role of the extracellular matrix (ECM) in cardiovascular homeostasis and disease. We are pursuing our studies using mouse models of Marfan syndrome (MFS), a multi-system disease caused by mutations in the ECM component and TGFβ regulator fibrillin-1, and a combination of genetic, pharmacological, ex vivo and computational approaches. Questions being addressed include the functional relationship between fibrillin-1 assemblies and muscle mechanosignaling as well as latent TGFβ bioavailability. Our long-term goal is to translate mouse findings into more effective treatment strategies for the life-threatening manifestations of MFS and related disease conditions.
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Luca Carta, Ph.D.
Silvia Smaldone, Ph.D.
Vesna Todorovic, Ph.D.
Josephine Galatioto, Ph.D.
Jason Cook, M.D., Ph.D
Maria del Solar, B.S.
Emily Chiu, B.A.
Rachel Zipursky, B.A.
- Cook J.R., Carta L., Benard L., Chemaly E.R., Chiu E., Rao S.K., Hampton T.G., Yurchenco P., Costa K.D., Hajjar R.J., Ramirez F. (2014) Abnormal muscle mechanosignaling triggers cardiomyopathy in mice with Marfan syndrome. J. Clin. Invest. 124:1329-1339. PMC Journal – In Process
- Mariko B., Pezet M., Escoubet B., Bouillot S., Andrieu JP., Starcher B., Quaglino E.D., Jacob M.P., Huber P. Ramirez F. and Faury G. (2011) Fibrillin-1 genetic deficiency leads to pathological aging of arteries in mice. J. Pathology 224:33-44. PMC3075583
- Nistala H., Lee-Arteaga S., Carta L., Cook J.R., Smaldone S., Siciliano G., Rifkin A.N., Dietz H.C., Rifkin D.B., and Ramirez F. (2010) Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome. Hum. Mol. Genet. 19:4790-4798. PMC2989889
- Carta, L., Wagenseil, J.E., Knutsen, R.H., Mariko, B., Faury, G., Davis, E.C., Starcher, B., Mecham, R.P. and Ramirez, F. (2009) Discrete contributions of elastic fiber components to arterial development and mechanical compliance. Arterioscler. Thromb. Vasc. Biol. 29:2083-2089. PMC36966
- Carta, L., Smaldone, S., Zilberberg, L., Loch, D., Dietz, H.C., Rifkin, D.B. and Ramirez, F. (2009) p38 MAPK is an early determinant of promiscuous Smad2/3 signaling in the aortas of fibrillin-1 (Fbn1)-null mice. J. Biol. Chem. 284:5630-5636. PMC2645821
- Ramirez, F. and Rifkin D.B. (2009) Extracellular microfibrils: contextual platforms for TGFb and BMP signaling. Curr. Opin. Cell Biol. 21:616-622. PMID: 19525102
- Ramirez, F. and Dietz H.C. (2009) Extracellular microfibrils in vertebrate development and disease processes. J. Biol. Chem. 284:14677-14681. PMID: 19349279