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 ...
Emad Gad, Ko Tsutsui, Douglas R Keene
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The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology. [PDF]
Int J Mol Sci, 2022 Fibrillin-1 is the major structural component of the 10 nm-diameter microfibrils that confer key physical and mechanical properties to virtually every tissue, alone and together with elastin in the elastic fibers.
Asano K +3 more
europepmc +5 more sources
POGLUT2 and POGLUT3 O-glucosylate multiple EGF repeats in fibrillin-1, -2, and LTBP1 and promote secretion of fibrillin-1. [PDF]
J Biol Chem, 2021 Fibrillin-1 (FBN1) is the major component of extracellular matrix microfibrils, which are required for proper development of elastic tissues, including the heart and lungs. Through protein-protein interactions with latent transforming growth factor (TGF) β-binding protein 1 (LTBP1), microfibrils regulate TGF-β signaling.
Williamson DB +3 more
europepmc +5 more sources
Succinylation Inhibits the Enzymatic Hydrolysis of the Extracellular Matrix Protein Fibrillin 1 and Promotes Gastric Cancer Progression. [PDF]
Adv Sci (Weinh), 2022 Extracellular matrix (ECM) remodeling is crucial in the regulation of gastric cancer (GC) progression. This work aims to reveal novel posttranslational modifications and their relevant mechanisms in GC. In 3D matrix culture and animal models, it is found
Wang X +7 more
europepmc +2 more sources
Fibrillin-1 deficiency in the outer perichondrium causes longitudinal bone overgrowth in mice with Marfan syndrome. [PDF]
Hum Mol Genet, 2022 A disproportionate tall stature is the most evident manifestation in Marfan syndrome (MFS), a multisystem condition caused by mutations in the extracellular protein and TGFβ modulator, fibrillin-1.
Sedes L +9 more
europepmc +2 more sources
Asprosin, a C-Terminal Cleavage Product of Fibrillin 1 Encoded by the FBN1 Gene, in Health and Disease. [PDF]
Mol Syndromol, 2022 Background: Asprosin is a novel fasting-induced, glucogenic, and orexigenic protein hormone that is discovered with the help of genetic studies in patients with neonatal progeroid syndrome.
Ovali MA, Bozgeyik I.
europepmc +2 more sources
The Fibrillin-1/VEGFR2/STAT2 signaling axis promotes chemoresistance via modulating glycolysis and angiogenesis in ovarian cancer organoids and cells. [PDF]
Cancer Commun (Lond), 2022 Chemotherapy resistance is a primary reason of ovarian cancer therapy failure; hence it is important to investigate the underlying mechanisms of chemotherapy resistance and develop novel potential therapeutic targets.
Wang Z +11 more
europepmc +2 more sources
Fibrillin-1-enriched microenvironment drives endothelial injury and vascular rarefaction in chronic kidney disease. [PDF]
Sci Adv, 2021 The study illustrates a crucial role of tissue microenvironment in mediating endothelial dropout and vascular rarefaction. Endothelial cell injury leading to microvascular rarefaction is a characteristic feature of chronic kidney disease (CKD).
Li L +11 more
europepmc +2 more sources
Arabidopsis fibrillin 1-2 subfamily members exert their functions via specific protein-protein interactions. [PDF]
J Exp Bot, 2022 Fibrillins (FBNs) are plastidial proteins found in photosynthetic organisms from cyanobacteria to higher plants. The function of most FBNs remains unknown. Here, we focused on members of the FBN subgroup comprising FBN1a, FBN1b, and FBN2.
Torres-Romero D +4 more
europepmc +2 more sources
Proof-of-Concept: Antisense Oligonucleotide Mediated Skipping of Fibrillin-1 Exon 52. [PDF]
Int J Mol Sci, 2021 Marfan syndrome is one of the most common dominantly inherited connective tissue disorders, affecting 2–3 in 10,000 individuals, and is caused by one of over 2800 unique FBN1 mutations.
Cale JM, Greer K, Fletcher S, Wilton SD.
europepmc +2 more sources