LTBPs in biology and medicine: LTBP diseases

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

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Bone Diseases / metabolism
  • Cardiovascular Diseases / metabolism
  • Extracellular Matrix / metabolism
  • Humans
  • Latent TGF-beta Binding Proteins / deficiency*
  • Latent TGF-beta Binding Proteins / metabolism*
  • Lung Diseases / metabolism
  • Signal Transduction
  • Transforming Growth Factor beta / metabolism*

Substances

  • Latent TGF-beta Binding Proteins
  • Transforming Growth Factor beta