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Encyclopedia > Fibrillin

Fibrillin is a protein, which is essential for the formation of elastic fibres found in connective tissue. Fibrillin is a major component of the microfibrils, that form a sheath surrounding the amorphous elastin. To date, 3 forms of fibrillin have been described. The fibrillin-1 protein had been 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 has been 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 arachnodactily. Recently, fibrillin-3 has been described and is believed to be located mainly in the brain. A representation of the 3D structure of myoglobin, showing coloured alpha helices. ...


  Results from FactBites:
 
Fibrillin - Wikipedia, the free encyclopedia (189 words)
Fibrillin is a glycoprotein, which is essential for the formation of elastic fibres found in connective tissue.
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.
Fbn2 - fibrillin 2 (642 words)
Of mice and Marfan: genetic linkage analyses of the fibrillin genes, Fbn1 and Fbn2, in the mouse genome.
Mutations of the mouse Twist and sy (fibrillin 2) genes induced by chemical mutagenesis of ES cells.
Fbn2 is a homologue of FBN2 (fibrillin 2 (congenital contractural arachnodactyly)) from Homo sapiens.
  More results at FactBites »


 

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