Microenvironmental regulation by fibrillin-1. [PDF]
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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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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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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Genome-Wide Identification and Expression Analyses of the Fibrillin Family Genes Suggest Their Involvement in Photoprotection in Cucumber [PDF]
Plants, 2018 Fibrillin (FBN) is a plastid lipid-associated protein found in photosynthetic organisms from cyanobacteria to plants. In this study, 10 CsaFBN genes were identified in genomic DNA sequences of cucumber (Chinese long and Gy14) through database searches ...
Inyoung Kim +5 more
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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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Involvement of Aquaporin 1 in the Motility and in the Production of Fibrillin 1 and Type I Collagen of Cultured Human Dermal Fibroblasts [PDF]
Cosmetics, 2022 Aminocarbonyl proteins increase with age in the dermal layer. Gene Chip analysis of mRNA expression in human dermal fibroblasts cultured on collagen gels treated with glyceraldehyde as an aminocarbonyl protein and on untreated collagen gels showed a ...
Kazuhisa Maeda, Shiori Yoshida
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Biogenesis and function of fibrillin assemblies [PDF]
Cell and Tissue Research, 2009 Fibrillin-1 and fibrillin-2 are large cysteine-rich glycoproteins that serve two key physiological functions: as supporting structures that impart tissue integrity and as regulators of signaling events that instruct cell performance. The structural role of fibrillins is exerted through the temporal and hierarchical assembly of microfibrils and elastic ...
Francesco Ramirez
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The Biomechanics of Fibrillin Microfibrils: Lessons from the Ciliary Zonule [PDF]
CellsMarfan syndrome is an inherited connective tissue disorder that affects the cardiovascular, musculoskeletal, and ocular systems. It is caused by pathogenic variants in the fibrillin-1 gene (FBN1).
Pooja Rathaur +6 more
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Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia – a novel mouse model providing insights into geleophysic dysplasia [PDF]
, 2015 Mutations in the secreted glycoprotein ADAMTSL2 cause recessive geleophysic dysplasia (GD) in humans and Musladin–Lueke syndrome (MLS) in dogs. GD is a severe, often lethal, condition presenting with short stature, brachydactyly, stiff skin, joint ...
Dirk Hubmacher +4 more
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