Results 51 to 60 of about 7,077 (251)
Fibrillin-1 Misfolding and Disease
Antioxidants & Redox Signaling, 2006 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
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Fibrillin-1 regulates the bioavailability of TGFβ1 [PDF]
The 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.
Cay M. Kielty +7 more
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Frontiers in Physiology, 2016 A characteristic feature of liver cirrhosis is the accumulation of large amounts of connective tissue with the prevailing content of type I collagen. Elastin is a minor connective tissue component in normal liver but it is actively synthesized by hepatic
Jiri Kanta
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Molecular assembly and mechanical properties of the extracellular matrix: A fibrous protein perspective [PDF]
, 2013 The extracellular matrix is an integral and dynamic component of all tissues. Macromolecular compositions and structural architectures of the matrix are tissue-specific and typically are strongly influenced by the magnitude and direction of biomechanical
Keeley, Fred W., Muiznieks, Lisa D.
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Aquaculture Reports, 2022 The razor clam, Sinonovacula constricta, must have acquired adaptive mechanisms to defense thermal pressure during evolution as it’s an eurythermal bivalve. However, the specific mechanisms remain unclear. To identify thermal responsive genes and unravel
Xianghui Kong +5 more
doaj
Putative functions of extracellular matrix glycoproteins in secondary palate morphogenesis
Frontiers in Physiology, 2012 Cleft palate is a common birth defect in humans. Elevation and fusion of paired palatal shelves are coordinated by growth and transcription factors, and mutations in these can cause malformations.
Rocca ed'Amaro +6 more
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Dual role for the latent transforming growth factor-beta binding protein in storage of latent TGF-beta in the extracellular matrix and as a structural matrix protein [PDF]
, 1995 The role of the latent TGF-beta binding protein (LTBP) is unclear. In cultures of fetal rat calvarial cells, which form mineralized bonelike nodules, both LTBP and the TGF-beta 1 precursor localized to large fibrillar structures in the extracellular ...
Bonewald, L.F. +4 more
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Journal of Biological Chemistry, 2003 Fibrillins are microfibril-forming extracellular matrix macromolecules that modulate skeletal development. In humans, mutations in fibrillins result in long bone overgrowth as well as other distinct phenotypes.
N. Charbonneau +6 more
semanticscholar +1 more source
Cell adhesion and integrin binding to recombinant human fibrillin-1 [PDF]
, 1996 Fibrillin-1 is a major constituent of tissue microfibrils that occur in most connective tissues, either in close association with or independent of elastin.
Pfaff, Martin +3 more
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Journal of Biological Chemistry, 2004 The interactions of microfibril-associated glycoprotein (MAGP)-2 have been investigated with fibrillins and fibrillin-containing microfibrils. Solid phase binding assays were conducted with recombinant fragments covering fibrillin-1 and most of fibrillin-
E. Hanssen +3 more
semanticscholar +1 more source