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 aspect== Mutations in FBN1 and FBN2 are...
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<protein> Widely distributed connective tissue protein (350 kD) associated with microfibrils (10nm diameter). ... (12 Nov 1997) ...
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Widely-distributed connective tissue protein (350 kD) associated with microfibrils (10nm diameter).
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Type: Term Pronunciation: fī′bril-in Definitions: 1. A microfibrillar protein in connective tissue with a wide distribution in the body; molecular weight about 350,000. There is good evidence that Marfan syndrome is due to mutations of fibrillin.
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fibrillin A protein constituent of connective tissue. It is present in skin, ligaments, tendons, and in the aorta.
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