Fibrillin-3 expression in human development |
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Authors: | Laetitia SabatierNicolai Miosge Dirk HubmacherGuoqing Lin Elaine C DavisDieter P Reinhardt |
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Institution: | a Faculty of Medicine, Department of Anatomy and Cell Biology, McGill University, Montreal, QC H3A 2B2, Canadab Faculty of Dentistry, McGill University, Montreal, QC H3A 2B2, Canadac Tissue Regeneration Work Group, Department of Prosthodontics, Georg August University, 37075 Göttingen, Germanyd Cincinnati Children''s Hospital Medical Center, Division of Developmental Biology, Cincinnati, OH 45229, USA |
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Abstract: | Fibrillin proteins are the major components of extracellular microfibrils found in many connective tissues. Fibrillin-1 and fibrillin-2 are well studied and mutations in these proteins cause a number of fibrillinopathies including Marfan syndrome and congenital contractural arachnodactyly, respectively. Fibrillin-3 was more recently discovered and is much less well characterized. Fibrillin-1 is expressed throughout life, whereas fibrillins-2 and -3 are thought to be primarily present during development. Here, we report detailed fibrillin-3 expression patterns in early human development.A polyclonal antiserum against a C-terminal recombinant half of human fibrillin-3 was produced in rabbit. Anti-fibrillin-3 antibodies were affinity-purified and antibodies cross-reacting with the other fibrillins were removed by absorption resulting in specific anti-fibrillin-3 antibodies. Immunohistochemical analyses with these purified antibodies demonstrate that fibrillin-3 is temporally expressed in numerous tissues relatively evenly from the 6th to the 12th gestational week. Fibrillin-3 was found spatially expressed in perichondrium, perineurium, perimysium, skin, developing bronchi, glomeruli, pancreas, kidney, heart and testis and at the prospective basement membranes in developing epithelia and endothelia. Double immunohistochemical analyses showed that all fibrillins are globally expressed in the same organs, with a number of differences on the tissue level in cartilage, perichondrium and developing bronchi. These results suggest that fibrillin-3, compared to the other fibrillins, fulfills both overlapping and distinct functions in human development. |
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Keywords: | Fibrillin Microfibrils Development Connective tissue Immunohistochemistry Basement membranes |
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