Fibrillin-3 expression in human development
| Autor: | Guoqing Lin, Nicolai Miosge, Laetitia Sabatier, Dieter P. Reinhardt, Dirk Hubmacher, Elaine C. Davis |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2010 |
| Předmět: |
musculoskeletal diseases
Central Nervous System Pathology medicine.medical_specialty congenital hereditary and neonatal diseases and abnormalities Connective tissue macromolecular substances Fibrillins Cardiovascular System Article Basement Membrane Bone and Bones 03 medical and health sciences 0302 clinical medicine Antibody Specificity Pregnancy Peripheral Nervous System medicine Perichondrium Animals Humans Congenital contractural arachnodactyly skin and connective tissue diseases Muscle Skeletal Molecular Biology 030304 developmental biology 0303 health sciences Perimysium biology integumentary system Immune Sera Microfilament Proteins medicine.disease Embryo Mammalian 3. Good health medicine.anatomical_structure Cartilage Polyclonal antibodies 030220 oncology & carcinogenesis biology.protein Immunohistochemistry Female Rabbits Fibrillin |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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