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Published on: December 23, 2010
LTBPs in biology and medicine: LTBP diseases
Daniel B Rifkin1, William J Rifkin2, Lior Zilberberg3
1Department of Cell Biology, NYU School of Medicine, New York, NY, United States; Department of Medicine, NYU School of Medicine, New York, NY, United States.
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.
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