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In Vivo Studies of Mutant Fibrillin-1 Microfibrils [PDF]

open access: yesJournal of Biological Chemistry, 2010
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, 2011
Emilio Arteaga-Solis   +2 more
exaly  

Fibrillin-containing microfibrils: Structure and function in health and disease

International Journal of Biochemistry and Cell Biology, 1995
Cay M Kielty, C A Shuttleworth
exaly  

ADAMTS10 Protein Interacts with Fibrillin-1 and Promotes Its Deposition in Extracellular Matrix of Cultured Fibroblasts

Journal of Biological Chemistry, 2011
Lauren W Wang   +2 more
exaly  

Microfibril Structure Masks Fibrillin-2 in Postnatal Tissues

Journal of Biological Chemistry, 2010
Noe L Charbonneau   +2 more
exaly  

Microenvironmental regulation by fibrillin-1. [PDF]

open access: yesPLoS 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 ...
Gerhard Sengle   +14 more
doaj   +5 more sources

Fibrillin-1 Regulates Arteriole Integrity in the Retina

open access: yesBiomolecules, 2022
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]

open access: yesJournal 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.
Stuart A Cain   +2 more
exaly   +4 more sources

Fibrillin-1 regulates endothelial sprouting during angiogenesis

open access: yesProceedings of the National Academy of Sciences of the United States of America, 2023
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

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