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In Vivo Studies of Mutant Fibrillin-1 Microfibrils [PDF]
In humans, mutations in fibrillin-1 result in a variety of genetic disorders with distinct clinical phenotypes. While most of the known mutations in fibrillin-1 cause Marfan syndrome, a number of other mutations lead to clinical features unrelated to Marfan syndrome. Pathogenesis of Marfan syndrome is currently thought to be driven by mechanisms due to
Noe L Charbonneau +2 more
exaly +3 more sources
Some of the next articles are maybe not open access.
Material and mechanical properties of bones deficient for fibrillin-1 or fibrillin-2 microfibrils
Matrix Biology, 2011Emilio Arteaga-Solis +2 more
exaly
Fibrillin-containing microfibrils: Structure and function in health and disease
International Journal of Biochemistry and Cell Biology, 1995Cay M Kielty, C A Shuttleworth
exaly
The fibrillin microfibril scaffold: A niche for growth factors and mechanosensation?
Matrix Biology, 2015Gerhard Sengle, Lynn Y Sakai
exaly
Microfibril Structure Masks Fibrillin-2 in Postnatal Tissues
Journal of Biological Chemistry, 2010Noe L Charbonneau +2 more
exaly
Microenvironmental regulation by fibrillin-1. [PDF]
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
doaj +5 more sources
Fibrillin-1 Regulates Arteriole Integrity in the Retina
Fibrillin-1 is an extracellular matrix protein that assembles into microfibrils that provide critical functions in large blood vessels and other tissues.
Florian Alonso +4 more
doaj +4 more sources
Fibrillin-1 regulates the bioavailability of TGFβ1 [PDF]
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.
Stuart A Cain +2 more
exaly +4 more sources
Fibrillin-1 regulates endothelial sprouting during angiogenesis
Fibrillin-1 is an extracellular matrix protein that assembles into microfibrils which provide critical functions in large blood vessels and other tissues.
Florian Alonso +2 more
exaly +4 more sources

