FIBRILLINS IN TENDON
| Autor: | Guglielmina Pepe, Betti Giusti |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
Marfan syndrome musculoskeletal diseases Aging congenital hereditary and neonatal diseases and abnormalities Cognitive Neuroscience Mini Review Connective tissue macromolecular substances fibrillin Marfan Syndrome lcsh:RC321-571 Extracellular matrix 03 medical and health sciences medicine Oxytalan fibres skin and connective tissue diseases lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry Tendon Muscle contracture biology integumentary system business.industry elastic fibers Contractures Elastic fibers Fibrillin Oxytalan fibers Fibrillins Anatomy medicine.disease musculoskeletal system contractures Extracellular Matrix Elastic fibres 030104 developmental biology medicine.anatomical_structure biology.protein oxytalan fibers business Elastin Neuroscience |
| Zdroj: | Frontiers in Aging Neuroscience, Vol 8 (2016) Frontiers in Aging Neuroscience |
| ISSN: | 1663-4365 |
| DOI: | 10.3389/fnagi.2016.00237/full |
| Popis: | Tendons among connective tissue, mainly collagen, contain also elastic fibres made of fibrillin 1, fibrillin 2 and elastin that are broadly distributed in tendons and represent 1-2% of the dried mass of the tendon. Only in the last years, studies on structure and function of elastic fibres in tendons have been performed. Aim of this review is to revise data on the organization of elastic fibres in tendons, in particular fibrillin structure and function, and on the clinical manifestations associated to alterations of elastic fibres in tendons. Indeed, microfibrils may contribute to tendon mechanics; therefore, their alterations may cause joint hypermobility and contractures which have been found to be clinical features in patients with Marfan syndrome and Beals syndrome. The two diseases are caused by mutations in genes FBN1 and FBN2 encoding fibrillin 1 and fibrillin 2, respectively. |
| Databáze: | OpenAIRE |
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