Results 11 to 20 of about 12,218 (212)
Microenvironmental regulation by fibrillin-1. [PDF]
Fibrillin-1 is a ubiquitous extracellular matrix molecule that sequesters latent growth factor complexes. A role for fibrillin-1 in specifying tissue microenvironments has not been elucidated, even though the concept that fibrillin-1 provides ...
Gerhard Sengle +14 more
doaj +5 more sources
The Diversity of Fibrillin Functions: Lessons from the Periodontal Ligament [PDF]
Marfan syndrome is caused by a mutation in the FBN1 gene encoding fibrillin-1. This extracellular matrix glycoprotein, which assembles into microfibrils, is best known for its scaffolding role in the production of elastic fibers responsible for ...
Elisabeth Genot +4 more
doaj +3 more sources
Fibrillin-1 Regulates Arteriole Integrity in the Retina
Fibrillin-1 is an extracellular matrix protein that assembles into microfibrils that provide critical functions in large blood vessels and other tissues.
Florian Alonso +4 more
doaj +2 more sources
Tendons among connective tissue, mainly collagen, contain also elastic fibres made of fibrillin 1, fibrillin 2 and elastin that are broadly distributed in tendons and represent 1-2% of the dried mass of the tendon.
Betti Giusti, Guglielmina Pepe
doaj +4 more sources
Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and nonelastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for mature elastic fibers.
Kielty, Cay M. +3 more
openaire +5 more sources
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 beta binding protein-like (TB) domains.
Whiteman, P, Hutchinson, S, Handford, P
openaire +4 more sources
Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development
The embryonic extracellular matrix (ECM) undergoes transition to mature ECM as development progresses, yet few mechanisms ensuring ECM proteostasis during this period are known.
Timothy J Mead +10 more
doaj +2 more sources
Fibrillin–integrin interactions in health and disease
Human fibrillin-1 is the major structural protein of extracellular matrix 10–12 nm microfibrils. It has a disulfide-rich modular organization which consists primarily of cbEGF (Ca2+-binding epidermal growth factor-like) domains and TB (transforming growth factor β-binding protein-like) domains.
Jovanovic, J +4 more
openaire +5 more sources
Arabidopsis thaliana Fibrillin 3a binds saturated fatty acids of chloroplast membranes [PDF]
Fibrillins (FBNs) are conserved plastid lipid–associated proteins involved in lipid storage, stress adaptation and plastid ultrastructure. While several Arabidopsis thaliana FBNs have been functionally characterized, the biochemical properties of the ...
Andrés Herrera-Tequia +2 more
doaj +2 more sources
The Supramolecular Organization of Fibrillin-Rich Microfibrils [PDF]
We propose a new model for the alignment of fibrillin molecules within fibrillin microfibrils. Automated electron tomography was used to generate three-dimensional microfibril reconstructions to 18.6-Å resolution, which revealed many new organizational details of untensioned microfibrils, including heart-shaped beads from which two arms emerge, and ...
Baldock, Clair +7 more
openaire +6 more sources

