Multi-organ phenotypes in mice lacking latent TGFβ binding protein 2 (LTBP2)

Dev Dyn. 2024 Feb;253(2):233-254. doi: 10.1002/dvdy.651. Epub 2023 Sep 9.

Abstract

Background: Latent TGFβ binding protein-2 (LTBP2) is a fibrillin 1 binding component of the microfibril. LTBP2 is the only LTBP protein that does not bind any isoforms of TGFβ, although it may interfere with the function of other LTBPs or interact with other signaling pathways.

Results: Here, we investigate mice lacking Ltbp2 (Ltbp2-/- ) and identify multiple phenotypes that impact bodyweight and fat mass, and affect bone and skin development. The alterations in skin and bone development are particularly noteworthy since the strength of these tissues is differentially affected by loss of Ltbp2. Interestingly, some tissues that express high levels of Ltbp2, such as the aorta and lung, do not have a developmental or homeostatic phenotype.

Conclusions: Analysis of these mice show that LTBP2 has complex effects on development through direct effects on the extracellular matrix (ECM) or on signaling pathways that are known to regulate the ECM.

Keywords: LTBP2; LTBPs; TGFβ; fibroblasts; myofibroblasts.

Publication types

  • Review

MeSH terms

  • Animals
  • Carrier Proteins* / genetics
  • Carrier Proteins* / metabolism
  • Extracellular Matrix* / metabolism
  • Mice
  • Phenotype
  • Protein Binding
  • Protein Isoforms / metabolism
  • Transforming Growth Factor beta / metabolism

Substances

  • Carrier Proteins
  • Transforming Growth Factor beta
  • Protein Isoforms