Complex contributions of fibronectin to initiation and maturation of microfibrils
| Autor: | Christine Fagotto-Kaufmann, Marian Chen, Douglas S. Annis, Deane F. Mosher, Laetitia Sabatier, Dieter P. Reinhardt, Jelena Djokic |
|---|---|
| Rok vydání: | 2013 |
| Předmět: |
musculoskeletal diseases
congenital hereditary and neonatal diseases and abnormalities Adolescent Fibrillin-1 Connective tissue Biology Fibrillins Binding Competitive Biochemistry Article Protein Interaction Mapping medicine Homeostasis Humans Protein Interaction Domains and Motifs Adhesins Bacterial Child skin and connective tissue diseases Molecular Biology Cells Cultured Binding Sites integumentary system Microfilament Proteins Infant Cell Biology Microfilament Protein Fibronectins Cell biology Fibronectin Protein Transport medicine.anatomical_structure Child Preschool Microfibrils Ultrastructure biology.protein Microfibril Protein Multimerization Fibrillin |
| Zdroj: | Biochemical Journal. 456:283-295 |
| ISSN: | 1470-8728 0264-6021 |
| DOI: | 10.1042/bj20130699 |
| Popis: | Fibrillins constitute the backbone of extracellular multifunctional assemblies present in elastic and non-elastic matrices, termed microfibrils. Assembly of fibrillins into microfibrils and their homoeostasis is poorly understood and is often compromised in connective tissue disorders such as Marfan syndrome and other fibrillinopathies. Using interaction mapping studies, we demonstrate that fibrillins require the complete gelatin-binding region of fibronectin for interaction, which comprises domains FNI6–FNI9. However, the interaction of fibrillin-1 with the gelatin-binding domain of fibronectin is not involved in fibrillin-1 network assembly mediated by human skin fibroblasts. We show further that the fibronectin network is essential for microfibril homoeostasis in early stages. Fibronectin is present in extracted mature microfibrils from tissue and cells as well as in some in situ microfibrils observed at the ultrastructural level, indicating an extended mechanism for the involvement of fibronectin in microfibril assembly and maturation. |
| Databáze: | OpenAIRE |
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