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Article

Fibrillin-1 Misfolding and Disease

1 Mar 2006

Abstract excerpt

Fibrillin-1 is a 350 kDa calcium-binding protein which assembles to form 10-12 nm microfibrils in the extracellular matrix (ECM). The structure of fibrillin-1 is dominated by two types of disulfide-rich motifs, the calcium- binding epidermal growth factor-like (cbEGF) and transforming growth factor beta binding protein-like (TB) domains. Disruption of fibrillin-1 domain structure and function contributes to the...

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Identifiers

PMID: 16677079
DOI: 10.1089/ars.2006.8.338

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