1. Fibrillin microfibrils and elastic fibre proteins: Functional interactions and extracellular regulation of growth factors.
- Author
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Thomson, Jennifer, Singh, Mukti, Eckersley, Alexander, Cain, Stuart A., Sherratt, Michael J., and Baldock, Clair
- Subjects
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FIBRILLIN , *GROWTH factors , *PROTEIN-protein interactions , *MICROFIBRILS , *OBSTRUCTIVE lung diseases - Abstract
Abstract Fibrillin microfibrils are extensible polymers that endow connective tissues with long-range elasticity and have widespread distributions in both elastic and non-elastic tissues. They act as a template for elastin deposition during elastic fibre formation and are essential for maintaining the integrity of tissues such as blood vessels, lung, skin and ocular ligaments. A reduction in fibrillin is seen in tissues in vascular ageing, chronic obstructive pulmonary disease, skin ageing and UV induced skin damage, and age-related vision deterioration. Most mutations in fibrillin cause Marfan syndrome, a genetic disease characterised by overgrowth of the long bones and other skeletal abnormalities with cardiovascular and eye defects. However, mutations in fibrillin and fibrillin-binding proteins can also cause short-stature pathologies. All of these diseases have been linked to dysregulated growth factor signalling which forms a major functional role for fibrillin. [ABSTRACT FROM AUTHOR]
- Published
- 2019
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