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Frontiers in Aging Neuroscience, 2016 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
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Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development
Matrix Biology, 2002 The extracellular glycoproteins fibrillin-1 and fibrillin-2 are major components of connective tissue microfibrils. Mutations in the fibrillin-1 and fibrillin-2 genes are responsible for the phenotypical manifestations of Marfan syndrome and congenital contractural arachnodactyly respectively, which emphasizes their essential roles in developmental ...
Dieter Peter Reinhardt +2 more
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Microenvironmental regulation by fibrillin-1.
PLoS Genetics, 2012 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
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The Diversity of Fibrillin Functions: Lessons from the Periodontal Ligament [PDF]
CellsMarfan 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
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Material and mechanical properties of bones deficient for fibrillin-1 or fibrillin-2 microfibrils [PDF]
Matrix Biology, 2011 The contribution of non-collagenous components of the extracellular matrix to bone strength is largely undefined. Here we report that deficiency of fibrillin-1 or fibrillin-2 microfibrils causes distinct changes in bone material and mechanical properties. Morphometric examination of mice with hypomorphic or null mutations in fibrillin-1 or fibrillin-2,
Mitchell Schaffler +2 more
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Fibrillin-1 regulates the bioavailability of TGFβ1 [PDF]
Journal of Cell Biology, 2007 We have discovered that fibrillin-1, which forms extracellular microfibrils, can regulate the bioavailability of transforming growth factor (TGF) β1, a powerful cytokine that modulates cell survival and phenotype. Altered TGFβ signaling is a major contributor to the pathology of Marfan syndrome (MFS) and related diseases.
Stuart Cain, Sarah Dallas, Cay Kielty
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Arabidopsis thaliana Fibrillin 3a binds saturated fatty acids of chloroplast membranes [PDF]
Plant Signaling & BehaviorFibrillins (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
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Macromolecular crowding enhances fibrillin-1 deposition in the extracellular matrix [PDF]
European Cells & Materials, 2022 Biochemical and biophysical factors need consideration when modelling in vivo cellular behaviour using in vitro cell culture systems. One underappreciated factor is the high concentration of macromolecules present in vivo, which is typically not ...
B Satz-Jacobowitz +3 more
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Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development
eLife, 2022 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
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Circulating fibrillin fragment concentrations in patients with and without aortic pathology
JVS - Vascular Science, 2022 Objective: Fragments of fibrillin-1 and fibrillin-2 will be detectable in the plasma of patients with aortic dissections and aneurysms. We sought to determine whether the plasma fibrillin fragment levels (PFFLs) differ between patients with thoracic ...
Eric J. Carlson, PhD +11 more
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