Specificity of latent TGF-β binding protein (LTBP) incorporation into matrix: role of fibrillins and fibronectin.

ABSTRACT: Fibrillin microfibrils are extracellular matrix structures with essential functions in the development and the organization of tissues including blood vessels, bone, limbs and the eye. Fibrillin-1 and fibrillin-2 form the core of fibrillin microfibrils, to which multiple proteins associate to form a highly organized structure. Defining the components of this structure and their interactions is crucial to understand the pathobiology of microfibrillopathies associated with mutations in fibrillins and in microfibril-associated molecules. In this study, we have analyzed both in vitro and in vivo the role of fibrillin microfibrils in the matrix deposition of latent TGF-β binding protein 1 (LTBP-1), -3 and -4; the three LTBPs that form a complex with TGF-β. In Fbn1(-/-) ascending aortas and lung

SUBMITTER: Zilberberg L 

PROVIDER: S-EPMC3404192 | biostudies-literature | 2012 Dec

REPOSITORIES: biostudies-literature