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(fī'bril-in), [MIM*134797]
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.
[Mod. L. fibrilla, fibril, + -in]


Etymology: L, fibrilla, small fiber
a major component of elastin-associated microfibrils. It is linked to Marfan's syndrome by findings of immunohistochemical studies and is associated with arachnodactyly, a disease similar to Marfan's syndrome.


An extracellular matrix protein, a major component of microfibrils associated with elastin fibres. People with MARFAN'S SYNDROME have abnormal fibrillin as a result of mutations of the FBN1 gene on chromosome 15.


A protein that is an important part of the structure of the body's connective tissue. In Marfan's syndrome, the gene responsible for fibrillin has mutated, causing the body to produce a defective protein.
Mentioned in: Marfan Syndrome
References in periodicals archive ?
4 Human genes: CBS, cystathionine-beta-synthase; MTRR, 5-methyltetrahydrofolate-homocysteine methyltransferase reductase; MTR, 5-methyltetrahydrofolate-homocysteine methyltransferase; MMACHC, methylmalonic aciduria (cobalamin deficiency) cblC type, with homocystinuria; MMADHC, methylmalonic aciduria (cobalamin deficiency) cblD type, with homocystinuria; LMBRD1, LMBR1 domain containing 1; FBN1, fibrillin 1.
It is important for parents to know that nothing that they did caused the fibrillin gene to mutate.
Study results showed that pro-collagen type I content increased by 65%, elastin by 18% and fibrillin by 6% after 8 weeks of Verisol treatment.
Hollister at the University of Nebraska Medical Center in Omaha attributed Marfan's to a defect in fibrillin, the protein that provides the structural scaffolding between cells (SN: 8/4/90, p.
Rich in sulfated galactans derived from the Hypnea musciformis algae, this novel ingredient is said to protect the cytoskeleton by maintaining vimentin organization and preserving synthesis of the extracellular matrix proteins; as well as support the matrix network by stimulating the formation of collagen and fibrillin networks.
elastin and fibrillin at the beginning of the treatment, and after 8 weeks of intake.
The BBC's Horizon programme highlighted a study which showed the original cream, the No7 Protect & Perfect Beauty Serum, stimulated the production of fibrillin, a protein that boosts skin's elasticity.
BBC Horizon approached a team of scientists at Manchester University who were conducting research into skin aging, especially the effect of UV light on structures like fibrillin that provides the skin with mechanical support.
Identification of defects in the fibrillin gene and protein in individuals with the Marfan syndrome and related disorders.
Marfan syndrome is caused by a defect in the gene that determines the structure of fibrillin, a protein that is an important part of connective tissue.
People with Marfan's syndrome lack sufficient amounts of a widely distributed body protein called fibrillin, according to Pyeritz, David W.
Acute IR irradiation also increases new, leaky vessel formation and induces inflammatory cellular infiltration and (b) Heat energy, which increases skin temperature, also increases MMP-1, -3, and -12, and modulates elastin and Fibrillin synthesis, resulting in the development of solar elastosis.