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.
journal_name
Semin Cell Dev Bioljournal_title
Seminars in cell & developmental biologyauthors
Thomson J,Singh M,Eckersley A,Cain SA,Sherratt MJ,Baldock Cdoi
10.1016/j.semcdb.2018.07.016subject
Has Abstractpub_date
2019-05-01 00:00:00pages
109-117eissn
1084-9521issn
1096-3634pii
S1084-9521(18)30018-1journal_volume
89pub_type
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