Microenvironmental Regulation by Fibrillin-1
نویسندگان
چکیده
منابع مشابه
Microenvironmental Regulation by Fibrillin-1
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 extracellular control of growth factor signaling is currently appreciated. Mutations in FBN1 are mainly responsible for the Marfan syndrome (MFS), recogni...
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We have discovered that fibrillin-1, which forms extracellular microfibrils, can regulate the bioavailability of transforming growth factor (TGF) beta1, a powerful cytokine that modulates cell survival and phenotype. Altered TGFbeta signaling is a major contributor to the pathology of Marfan syndrome (MFS) and related diseases. In the presence of cell layer extracellular matrix, a fibrillin-1 s...
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ژورنال
عنوان ژورنال: PLoS Genetics
سال: 2012
ISSN: 1553-7404
DOI: 10.1371/journal.pgen.1002425