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Microenvironmental Regulation by Fibrillin-1

Microenvironmental Regulation by Fibrillin-1
Sengle G, Tsutsui K, Keene DR, Tufa SF, Carlson EJ, Charbonneau NL, et al. (2012) Microenvironmental Regulation by Fibrillin-1. PLoS Genet 8(1): e1002425. https://doi.org/10.1371/journal.pgen.1002425 Fibrillin-1 is a ubiquitous extracellular matrix molecule that sequesters latent growth factor complexes.
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Interaction of fibrillin-1 fragments with transforming growth factor 1

, 2012
Fibrillins are large structural glycoproteins in the extracellular matrix that multimerize to form microfibril suprastructures in connective tissues. Fibrillin-containing microfibrils confer mechanical stability to tissues and regulate bioavailability of
Kaur, Jasvir
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ADAMTSL-6 Is a Novel Extracellular Matrix Protein That Binds to Fibrillin-1 and Promotes Fibrillin-1 Fibril Formation

ADAMTSL-6 Is a Novel Extracellular Matrix Protein That Binds to Fibrillin-1 and Promotes Fibrillin-1 Fibril Formation
This research was originally published in the Journal of Biological Chemistry. Ko Tsutsui, Ri-ichiroh Manabe, Tomiko Yamada, Itsuko Nakano, Yasuko Oguri, Douglas R. Keene, Gerhard Sengle, Lynn Y. Sakai and Kiyotoshi Sekiguchi. ADAMTSL-6 Is a Novel Extracellular Matrix Protein That Binds to Fibrillin-1 and Promotes Fibrillin-1 Fibril Formation. J. Biol.
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Defective Calcium Binding to Fibrillin-1: Consequence of an N2144S Change for Fibrillin-1 Structure and Function

Journal of Molecular Biology, 1999
Fibrillin-1 is a major structural component of 10-12 nm connective tissue microfibrils and has a modular organisation that includes 43 calcium binding epidermal growth factor-like (cbEGF) domains and seven transforming growth factor beta-binding protein-like (TB) domains.
S, Kettle, X, Yuan, G, Grundy, V, Knott, A K, Downing, P A, Handford   +5 more
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Fibrillin‐1 in the Vasculature: In Vivo Accumulation of eGFP‐Tagged Fibrillin‐1 in a Knockin Mouse Model

The Anatomical Record, 2019
ABSTRACTImmunolocalization studies have shown that fibrillin‐1 is distributed ubiquitously in the connective tissue space from early embryonic times through old age. When mutated, the gene for fibrillin‐1 (FBN1) causes the Marfan syndrome, a common inherited disorder of connective tissue.
Noe L. Charbonneau, Elise C. Manalo, Sara F. Tufa, Eric J. Carlson, Valerie M. Carlberg, Douglas R. Keene, Lynn Y. Sakai   +6 more
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Non-Enzymatic Glycation of Human Fibrillin-1

Gerontology, 2008
Non-enzymatic glycation of proteins is one of the key mechanisms in the pathogenesis of diabetic complications and may be significant in the age-related changes of tissues. We isolated and investigated the in vitro glycation of human aortic fibrillin-1. Fibrillin-1 was prepared from thoracic aortas of 9 accident victims distributed in three age groups.
Milena, Atanasova, Emiliana, Konova, Tatiana, Betova, Stephan, Baydanoff   +3 more
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Regulation of fibrillin-1 gene expression by Sp1

Gene, 2013
Mutations in the fibrillin-1 gene (FBN1) cause Marfan Syndrome (MFS), a hereditary disorder of connective tissue. The transcription of FBN1 has been reported to be driven by a short ultraconserved region (SUPR) in the 5' untranslated exon A of FBN1, but the nature of other factors involved in FBN1 gene regulation has not been clarified.
Gao, Guo, Christian, Rödelsperger, Martin, Digweed, Peter N, Robinson   +3 more
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Fibrillin-1 and asprosin, novel players in metabolic syndrome

Molecular Genetics and Metabolism, 2023
Fibrillin-1 is a major component of the extracellular microfibrils, where it interacts with other extracellular matrix proteins to provide elasticity to connective tissues, and regulates the bioavailability of TGFβ family members. A peptide consisting of the C-terminal 140 amino acids of fibrillin-1 has recently been identified as a glucogenic hormone,
Summers, Kim M., Bush, Stephen J., Davis, Margaret R., Hume, David A., Keshvari, Sahar, West, Jennifer A.   +5 more
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PARTIAL CLONING AND SEQUENCING OF CHICK FIBRILLIN-1 cDNA

In Vitro Cellular & Developmental Biology - Animal, 2000
The recent identification of numerous matrix genes and gene products has allowed a detailed examination of their roles in development. Two of these extracellular matrix proteins, fibrillin-1 and fibrillin-2, are components of the elastin-associated microfibrils.
G, Zhou, C E, Price, T H, Rosenquist, P F, Gadson, M, Godfrey   +4 more
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Fibrillin-1 im Bindegewebe

2017
Bindegewebe bezeichnet verschiedene Gewebetypen im Korper, die auf unterstutzende Aufgaben spezialisiert sind. Diese Gewebetypen enthalten relativ wenige Korperzellen sowie das biologische Material zwischen den Zellen, die so genannte extrazellulare Matrix.
Karina A. Zeyer, Dieter P. Reinhardt
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