Results 121 to 130 of about 12,218 (212)
Fibrillin secretion and microfibril assembly by Marfan dermal fibroblasts
, 1994 The Marfan syndrome has been linked to the FBN1 gene encoding the microfibrillar glycoprotein fibrillin. To date, there have been no descriptions of microfibrillar abnormalities characteristic of this connective tissue disorder, although biochemical ...
Pope, F. Michael +4 more
core +1 more source
Heparan sulfate regulates fibrillin-1 N- and C-terminal interactions
, 2008 Fibrillin-1 N- and C- terminal heparin binding sites have been characterized. An unprocessed monomeric N-terminal fragment (PF1) induced a very high heparin binding response, indicating heparin-mediated multimerization.
Baldwin, Andrew K. +13 more
core +1 more source
Fibrillin assemblies: extracellular determinants of tissue formation and fibrosis
, 2010 The extracellular matrix (ECM) plays a key role in tissue formation, homeostasis and repair, mutations in ECM components have catastrophic consequences for organ function and therefore, for the fitness and survival of the organism.
Francesco Ramirez +2 more
core +1 more source
Oxytalan fibers are distributed in the eye and periodontal ligaments (PDL). The ciliary zonule, known as Zinn's zonule, in the eye is composed of oxytalan fibers, which are bundles of microfibrils consisting mainly of fibrillin-1 and fibrillin-2.
Oka, Kyoko +4 more
core
Fibrillin in the alveoli in lung tissue (human, adult)
, 2006 Stain: imunoperoxidase staining with anti-fibrillin antibodies and diaminobenzidin reaction (frozen section). Fibrillin is one of the elastin-associated microfibrillar proteins that wraps the elastin protein core and is localized in normal lung ...
Poels, Lambert G.
core
New insights into the structural role of EMILINs within the human skin microenvironment
Scientific ReportsSupramolecular extracellular matrix (ECM) networks play an essential role in skin architecture and function. Elastin microfibril interface-located proteins (EMILINs) comprise a family of three extracellular glycoproteins that serve as essential ...
Alvise Schiavinato +15 more
doaj +1 more source
Zdravniški Vestnik, 2005 Background: The Marfan syndrome is an autosomal dominant heritable disorder of connective tissue. It is caused by mutations in the fibrillin-1 gene encoding glycoprotein fibrillin-1, a component of microfibrils of extracellular matrix.
Galina Pungerčič
doaj
Fibrillin assembly: dimer formation mediated by amino-terminal sequences
, 1999 We have investigated recombinant fibrillin-1 (profib-1) and fibrillin-2 (glyfib-2) molecules encoding the proline- or glycine-rich regions with flanking domains (exons 9-11), in order to establish whether these sequences might mediate specific molecular ...
Richard Wilson (14753071) +4 more
core +1 more source
Could the Phenotypic Outcomes of Genetic Variability in Cells Operating in Mechanically Dynamic Environments be Influenced by a Disrupted "Cell-ECM" Relationship? Using Cystic Fibrosis and Marfan Syndrome as an Example. [PDF]
BioessaysHart DA.
europepmc +1 more source