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Review
. 2018 Oct:71-72:90-99.
doi: 10.1016/j.matbio.2017.11.014. Epub 2017 Dec 5.

LTBPs in biology and medicine: LTBP diseases

Daniel B Rifkin  1 , William J Rifkin  2 , Lior Zilberberg  3
Affiliations
Review

LTBPs in biology and medicine: LTBP diseases

Daniel B Rifkin et al. Matrix Biol. 2018 Oct.

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.

Keywords: Aneurysm; Elastin maturation; Fibrillin; Latent TGFβ binding proteins; Marfan syndrome; TGFβ; TGFβ activation.

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Figures

Figure 1
Figure 1
Graphic illustration of where on each of the four LTBPs a number of ECM proteins bind.
Figure 2
Figure 2
Illustration of synthesis, secretion, and activation of LTBP – TGF beta complexes. A. Synthesis, secretion and activation by integrins (left) or proteases (right) of latent TGF beta bound to an LTBP. B. Secretion of an LTBP interacting with matrix components in the assembly of elastin containing microfibrils.
See this image and copyright information in PMC

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