LTBPs in biology and medicine: LTBP diseases

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. •The latent TGF beta binding proteins (LTBPs) are important matrix mediators of biological functions.•These molecules are crucial components of the regulation of both TGF beta action and elastin organization.•This review describes the biology of the LTBPs and outlines the physiological and pathological consequences of LTBP activity/loss.•Special attention is paid to in vivo analysis.