Fibrillin
protein family
Fibrillin is a glycoprotein, which is essential for the formation of elastic fibers found in connective tissue.
Fibrillin is secreted into the extracellular matrix by fibroblasts and becomes incorporated into the insoluble microfibrils, which appear to provide a scaffold
for deposition of elastin.
Clinical aspects
Marfan syndrome is a genetic disorder of the connective tissue caused by defected FBN1 gene. Mutations in FBN1 and FBN2 are also sometimes associated with adolescent idiopathic scoliosis.
Types
Fibrillin-1
Fibrillin-1 is a major component of the microfibrils that form a sheath surrounding the amorphous elastin. It is believed that the microfibrils are composed of end-to-end polymers of fibrillin. To date, 3 forms of fibrillin have been described. The fibrillin-1 protein was isolated by Engvall in 1986, and mutations in the FBN1 gene cause Marfan syndrome.
This protein is found in humans, and its gene is found on chromosome 15. At present more than 1500 different mutations have been described.
“Fibrillin” enters the record as protein family. Crown Archives preserves that source wording while asking what Fibrillin, protein and family can confirm, complicate or overturn.
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“Fibrillin” is worth following because a concise public description often conceals a longer documentary argument. Here, Fibrillin, protein and family provides the most credible route into that argument.
Named sources, stable identifiers and responsible institutions provide the strongest route from overview to verifiable evidence. The source revision retrieved here is dated Feb 20, 2025. The linked authority identifier is Q2909678. The first chronological checks are 1986 and 1500.
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This entry incorporates text from “Fibrillin” on English Wikipedia. Contributors are listed in the page history. Text is available under the Creative Commons Attribution-ShareAlike 4.0 License. Selected authority identifiers and statements are retrieved from Wikidata under CC0; their references and qualifiers remain part of the verification path.