Fibrillin-1 misfolding and disease.
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...
Main Authors: | Whiteman, P, Hutchinson, S, Handford, P |
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Format: | Journal article |
Language: | English |
Published: |
2006
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