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Fibrillin-1, a calcium binding protein of extracellular matrix.

Abstract:

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 by multiple tandem repeats of the calcium binding epidermal growth factor-like (...

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Publication status:
Published

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Institution:
University of Oxford
Division:
MSD
Department:
Biochemistry
Role:
Author
Journal:
Biochimica et biophysica acta More from this journal
Volume:
1498
Issue:
2-3
Pages:
84-90
Publication date:
2000-12-01
DOI:
ISSN:
0006-3002
Language:
English
Keywords:
Pubs id:
pubs:99851
UUID:
uuid:053b2fc8-7d80-4e0b-ba92-d6b4cd62956e
Local pid:
pubs:99851
Source identifiers:
99851
Deposit date:
2012-12-19

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