Fibrillin

Fibrillin is a glycoprotein, which is essential for the formation of elastic fibers found in connective tissue.

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 Sakai in 1986, and mutations in the gene have been linked to the Marfan syndrome. At present more than 100 different mutations have been described.

Fibrillin-2
Fibrillin-2 was isolated in 1994 by Zhang and is thought to play a role in early elastogenesis. Mutations in the fibrillin-2 gene have been linked to arachnodactyly (which is also a clinical symptom of Marfan syndrome).

Fibrillin-3
More recently, fibrillin-3 was described and is believed to be located mainly in the brain. Along with in the brain, fibrillin 1 has been localized in the gonads and ovaries of field mice.