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Elastic fibres are essential extracellular matrix macromolecules comprising an elastin core surrounded by a mantle of fibrillin-rich microfibrils that endow connective tissues with the critical properties of elasticity and resilience.
Missense mutations in COL8A2, the gene encoding the alpha2 chain of type VIII collagen, cause two forms of corneal endothelial dystrophy.
The underlying pathogenesis of FECD and PPCD may be related to disturbance of the role of type VIII collagen in influencing the terminal differentiation of the neural crest derived corneal endothelial cell.
The Collagen Family: Structure, Assembly, and Organization in the Extracellular Matrix
Fibrillin-1 regulates the bioavailability of TGFβ1
- Shazia S Chaudhry, S. Cain, A. Morgan, S. Dallas, C. Shuttleworth, C. Kielty
- BiologyThe Journal of cell biology
- 29 January 2007
We have discovered that fibrillin-1, which forms extracellular microfibrils, can regulate the bioavailability of transforming growth factor (TGF) β1, a powerful cytokine that modulates cell survival…
Fibronectin regulates latent transforming growth factor-beta (TGF beta) by controlling matrix assembly of latent TGF beta-binding protein-1.
It is shown that fibronectin is critical for the incorporation of LTBP1 and transforming growth factor-beta (TGF beta) into the ECM of osteoblasts and fibroblasts and a novel role for fibronECTin in regulation of TGF beta viaLTBP1 interactions is suggested.
Vascular endothelial growth factor can signal through platelet-derived growth factor receptors
Evidence is provided that VEGF-A can stimulate platelet-derived growth factor receptors (PDGFRs), thereby regulating MSC migration and proliferation and mediate human dermal fibroblast migration.
Elastic fibres in health and disease
- Andrew K. Baldwin, Andreja Simpson, R. Steer, S. Cain, C. Kielty
- Biology, MedicineExpert Reviews in Molecular Medicine
- 1 August 2006
This review outlines the latest understanding of the composition and assembly of elastic fibres, and describes elastic fibre diseases and current therapeutic approaches.
Fibrillin degradation by matrix metalloproteinases: implications for connective tissue remodelling.
This is the first demonstration that fibrillin molecules andfibrillin-rich microfibrils are degraded by MMPs and that certain amino acid substitutions change the fragmentation patterns, which have important implications for physiological and pathological fibrillo catabolism and for loss of connective tissue elasticity in ageing and disease.
An elastin gene mutation producing abnormal tropoelastin and abnormal elastic fibres in a patient with autosomal dominant cutis laxa.
It is suggested that the mutant tropoelastin protein is synthesized, secreted and incorporated into the elastic matrix, where it alters the architecture of elastic fibres and reduces elastic recoil in affected tissues.
Assembly of fibrillin microfibrils governs extracellular deposition of latent TGFβ
Control of the bioavailability of the growth factor TGFβ is essential for tissue formation and homeostasis, yet precisely how latent TGFβ is incorporated into the extracellular matrix is unknown.…