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fibrillin-1

Known as: fbn1, fibrillin-1 (FBN1), Fibrillin-1 [Chemical/Ingredient] 
A fibrillin (FBN1) that functions as a structural support protein for MICROFIBRILS. It also regulates the maturation of OSTEOBLASTS by controlling… 
National Institutes of Health

Related topics

Related topics

22 relations
Cell AdhesionCell MovementExtracellular MatrixExtracellular Matrix Protein Gene

Narrower (2)

FBN1 protein, humanProfibrillin 1

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Review
2009
Review
2009
Biogenesis and function of fibrillin assemblies
Fibrillin-1 and fibrillin-2 are large cysteine-rich glycoproteins that serve two key physiological functions: as supporting… 
Highly Cited
2008
Highly Cited
2008
Targeting of Bone Morphogenetic Protein Growth Factor Complexes to Fibrillin*
Both latent transforming growth factor-β (TGF-β)-binding proteins fibrillins are components of microfibril networks, and both… 
Review
2008
Review
2008
Ehlers-Danlos syndromes and Marfan syndrome.
Highly Cited
2002
Highly Cited
2002
Homo- and Heterotypic Fibrillin-1 and -2 Interactions Constitute the Basis for the Assembly of Microfibrils*
Fibrillin-1 and fibrillin-2 constitute the backbone of extracellular filaments, called microfibrils. Fibrillin assembly involves… 
Highly Cited
2001
Highly Cited
2001
Interactions of Fibrillin-1 with Heparin/Heparan Sulfate, Implications for Microfibrillar Assembly*
Fibrillin-1 is a major constituent of the 10–12 nm extracellular microfibrils. Here we identify, characterize, and localize… 
Highly Cited
1996
Highly Cited
1996
Fibrillin-1: organization in microfibrils and structural properties.
To investigate the microfibrillar organization and structural properties of fibrillin-1, we produced overlapping recombinant… 
Highly Cited
1996
Highly Cited
1996
Mutation in fibrillin-1 and the Marfanoid-craniosynostosis (Shprintzen-Goldberg) syndrome
Recent reports have described a distinct and recurrent pattern of systemic malformation that associates craniosynostosis and… 
Highly Cited
1996
Highly Cited
1996
Fibrillin-1 and Fibulin-2 Interact and Are Colocalized in Some Tissues*
Microfibrils 10-12 nm in diameter are found in elastic and non-elastic tissues with fibrillin as a major component. Little is… 
Highly Cited
1995
Highly Cited
1995
The structure of a Ca2+-binding epidermal growth factor-like domain: Its role in protein-protein interactions
Highly Cited
1995
Highly Cited
1995
A mutation in FBN1 disrupts profibrillin processing and results in isolated skeletal features of the Marfan syndrome.
Dermal fibroblasts from a 13-yr-old boy with isolated skeletal features of the Marfan syndrome were used to study fibrillin… 
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