The objectives of Core B are to provide Imaging services, antibodies, and antibody production services to the individual components of the Program Project. At the request of the other Pis, Dr. Sakai will discuss experiments, devise appropriate experimental strategies and protocols, and then perform agreed upon services. Imaging Services: Transmission electron microscopy (including immunolocalization as well as standard ultrastructural imaging) and confocal microscopy are available to all of the Projects. Antibodies: The following well-characterized antibodies will be distributed to the Projects upon request: rabbit polyclonal antibodies specific for fibrillin-1, fibrillin-2, LTBP-1, and LTBP-4;monoclonal antibodies specific for fibrillin and for BMP-7. These antibodies are all suitable for use with mouse materials. In addition, the Core can provide monoclonal antibodies specific for flbrillin-1, fibrillin-2, and fibrillin-3, and for elastin, which react with human, bovine, and avian fibrillins. The Core has also produced and characterized monoclonal antibodies specific for human fibulin-4. Custom-made antibodies: Rabbit polyclonal or mouse monoclonal antibodies can be generated, in consultation with Project Pis. Project Pis will provide the immunogen and will also perform some assistance with antibody screening and characterization. Because commercial sources of pSmad 2/3 antibodies have been unreliable and inconsistent, we will synthesize phospho-Smad peptides to use as immunogens, generate and characterize our own reliable pSmad2/3 antibodies. Recombinant proteins: Limited supplies of recombinant proteins are also available. These include recombinant fibrillin-1 and fibrillin-2 polypeptides;recombinant LTBP-1, LTBP-2, LTBP-3 and LTBP-4 polypeptides;and recombinant BMP-7.
Core B supports the goals of the Program Project by providing specialized imaging expertise and instrumentation, as well as experience in interpretation of connective tissue structure, that is available in only a few places in the world. In addition, the collection of antibody reagents and immunochemical expertise relevant to the goals ofthe Program Project is outstanding. All Projects benefit from the services available.
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| 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: |
| 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 |
| Horiguchi, Masahito; Todorovic, Vesna; Hadjiolova, Krassimira et al. (2015) Abrogation of both short and long forms of latent transforming growth factor-? binding protein-1 causes defective cardiovascular development and is perinatally lethal. Matrix Biol 43:61-70 |
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