fibrillin 1 and fibrillin 2

What are fibrillin 1 and fibrillin 2?

These fibrillins are large glycoproteins coded for by a gene located on chromosome 15 (fibrillin-1) and chromosome 5 (fibrillin-2). They are secreted by fibroblasts into the extracellular matrix and become incorporated into the insoluble microfibrillar proteins that provide a scaffold for elastin deposition. Fibrillin can also be found as isolated bundles of microfibrils in the skin, blood vessels, and several other tissues. Abnormalities in fibrillin-1 are thought to cause Marfan’s syndrome (see also Chapter 55 : Heritable Connective Tissue Disease), whereas abnormalities in fibrillin-2 cause congenital contractural arachnodactyly.


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