Core B will provide imaging services and antibodies to the projects in the Program. Microfibril ultrastructure in tissuesfrom mouse models relevant to the Marfan syndrome will be evaluated by transmission electron microscopy, byimmunoelectron microscopy, and by laser confocal microscopy as required by the individual projects. Extracellularmatrix elaborated by cells in culture can be similarly evaluated. A novel method of fixing tissues using high pressurefreezing has been developed which allows improved visualization of cell-matrix interactions in the aorta. This methodwill be available for Project Investigators to better analyze aortae from fibrillin-1 mutant mice. Monoclonal andpolyclonal antibodies specific for fibrillins, for LTBPs, and for BMP-7 will be produced, tested, and provided to theProjects as needed. A newly produced and characterized pan-fibrillin monoclonal antibody is now available and shouldallow better immunohistochemical visualization of fibrillin in mice. In addition to providing these reagents andservices, Core B will produce and characterize new monoclonal antibodies specifically required to advance the ProgramProject.
| MacFarlane, Elena Gallo; Haupt, Julia; Dietz, Harry C et al. (2017) TGF-? Family Signaling in Connective Tissue and Skeletal Diseases. Cold Spring Harb Perspect Biol 9: |
| Bellini, C; Korneva, A; Zilberberg, L et al. (2016) Differential ascending and descending aortic mechanics parallel aneurysmal propensity in a mouse model of Marfan syndrome. J Biomech 49:2383-2389 |
| Smaldone, Silvia; Ramirez, Francesco (2016) Fibrillin microfibrils in bone physiology. Matrix Biol 52-54:191-197 |
| Lee, Jia-Jye; Galatioto, Josephine; Rao, Satish et al. (2016) Losartan Attenuates Degradation of Aorta and Lung Tissue Micromechanics in a Mouse Model of Severe Marfan Syndrome. Ann Biomed Eng 44:2994-3006 |
| Sakai, Lynn Y; Keene, Douglas R; Renard, Marjolijn et al. (2016) FBN1: The disease-causing gene for Marfan syndrome and other genetic disorders. Gene 591:279-291 |
| Walji, Tezin A; Turecamo, Sarah E; DeMarsilis, Antea J et al. (2016) Characterization of metabolic health in mouse models of fibrillin-1 perturbation. Matrix Biol 55:63-76 |
| Robertson, Ian B; Rifkin, Daniel B (2016) Regulation of the Bioavailability of TGF-? and TGF-?-Related Proteins. Cold Spring Harb Perspect Biol 8: |
| Sengle, Gerhard; Sakai, Lynn Y (2015) The fibrillin microfibril scaffold: A niche for growth factors and mechanosensation? Matrix Biol 47:3-12 |
| Zilberberg, Lior; Phoon, Colin K L; Robertson, Ian et al. (2015) Genetic analysis of the contribution of LTBP-3 to thoracic aneurysm in Marfan syndrome. Proc Natl Acad Sci U S A 112:14012-7 |
| Robertson, Ian B; Horiguchi, Masahito; Zilberberg, Lior et al. (2015) Latent TGF-?-binding proteins. Matrix Biol 47:44-53 |
Showing the most recent 10 out of 78 publications
