Article
Fibrillin-1, a calcium binding protein of extracellular matrix.
Biochimica et biophysica acta - 20 Dec 2000
Handford P A
Abstract excerpt
Fibrillin-1 is a large extracellular matrix glycoprotein which assembles to form 10-12 nm microfibrils in extracellular matrix. Mutations in the human fibrillin-1 gene (FBN-1) cause the connective tissue disease Marfan syndrome and related disorders, which are characterised by defects in the skeletal, cardiovascular and ocular systems of the body. Fibrillin-1 has a striking modular organisation which is dominated...
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