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The N-Terminal Region of Fibrillin-1 Mediates a Bipartite Interaction with LTBP1.

Abstract:

Fibrillin-1 (FBN1) mutations associated with Marfan syndrome lead to an increase in transforming growth factor β (TGF-β) activation in connective tissues resulting in pathogenic changes including aortic dilatation and dissection. Since FBN1 binds latent TGF-β binding proteins (LTBPs), the major reservoir of TGF-β in the extracellular matrix (ECM), we investigated the structural basis for the FBN1/LTBP1 interaction. We present the structure of a four-domain FBN1 fragment, EGF2-EGF3-Hyb1-cbEGF1...

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Publication status:
Published
Peer review status:
Peer reviewed
Version:
Publisher's version

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Publisher copy:
10.1016/j.str.2017.06.003

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Institution:
University of Oxford
Department:
Oxford, MSD, Biochemistry
Role:
Author
More by this author
Institution:
University of Oxford
Department:
Oxford, MSD, Biochemistry
Role:
Author
More by this author
Institution:
University of Oxford
Department:
Oxford, MSD, Biochemistry
Role:
Author
More by this author
Institution:
University of Oxford
Department:
Oxford, MSD, Biochemistry
Role:
Author
More by this author
Institution:
University of Oxford
Department:
Oxford, MSD, Biochemistry
Role:
Author
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Biotechnology and Biological Sciences Research Council More from this funder
Publisher:
Cell Press Publisher's website
Journal:
Structure Journal website
Volume:
25
Issue:
8
Pages:
1208–1221.e5
Publication date:
2017-06-29
Acceptance date:
2017-06-01
DOI:
EISSN:
1878-4186
ISSN:
0969-2126
Pubs id:
pubs:704518
URN:
uri:a5c89ef9-2467-4235-b550-6b2fc7516241
UUID:
uuid:a5c89ef9-2467-4235-b550-6b2fc7516241
Local pid:
pubs:704518
Paper number:
8

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