| Literature DB >> 29217273 |
Daniel B Rifkin1, William J Rifkin2, Lior Zilberberg3.
Abstract
The latent transforming growth factor (TGF) β binding proteins (LTBP) are crucial mediators of TGFβ function, as they control growth factor secretion, matrix deposition, presentation and activation. Deficiencies in specific LTBP isoforms yield discrete phenotypes representing defects in bone, lung and cardiovascular development mediated by loss of TGFβ signaling. Additional phenotypes represent loss of unique TGFβ-independent features of LTBP effects on elastogenesis and microfibril assembly. Thus, the LTBPs act as sensors for the regulation of both growth factor activity and matrix function.Entities:
Keywords: Aneurysm; Elastin maturation; Fibrillin; Latent TGFβ binding proteins; Marfan syndrome; TGFβ; TGFβ activation
Mesh:
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Year: 2017 PMID: 29217273 PMCID: PMC5988920 DOI: 10.1016/j.matbio.2017.11.014
Source DB: PubMed Journal: Matrix Biol ISSN: 0945-053X Impact factor: 11.583