Format

Send to

Choose Destination
Matrix Biol. 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.

Author information

1
Department of Cell Biology, NYU School of Medicine, New York, NY, United States; Department of Medicine, NYU School of Medicine, New York, NY, United States. Electronic address: daniel.rifkin@med.nyu.edu.
2
Department of Plastic Surgery, NYU School of Medicine, New York, NY, United States.
3
Department of Cell Biology, 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.

KEYWORDS:

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

PMID:
29217273
PMCID:
PMC5988920
DOI:
10.1016/j.matbio.2017.11.014
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Elsevier Science Icon for PubMed Central
Loading ...
Support Center