Journal
MATRIX BIOLOGY
Volume 71-72, Issue -, Pages 90-99Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.matbio.2017.11.014
Keywords
TGF beta; Latent TGF beta binding proteins; Fibrillin; Marfan syndrome; TGF beta activation; Aneurysm; Elastin maturation
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Funding
- NCI NIH HHS [R01 CA034282] Funding Source: Medline
- NHLBI NIH HHS [P01 HL134605] Funding Source: Medline
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The latent transforming growth factor (TGF) beta binding proteins (LTBP) are crucial mediators of TGF beta 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 TGF beta signaling. Additional phenotypes represent loss of unique TGF beta-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. (C) 2017 Elsevier B.V. All rights reserved.
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