Latent transforming growth factor-β binding protein 1 (LTBP1) – only one representative of various related proteins involved in the development of liver fibrosis?

Latent transforming growth factor-β binding proteins (LTBP) forms a subfamily of the extracellular proteins fibrillin 1 and fibrillin 2 which constitute the backbone of extracellular filaments. Common features of the highly conserved LTBP structure are the pattern of eight cysteine residues and epidermal growth factor (EGF)-like repeats. LTBP are known to be important for the folding and secretion of TGF-β, localization to the ECM and also activation process of TGF-β particularly during liver fibrosis. While different stages of these processes are still unknown further investigations concerning the molecular mechanisms are essential particularly with regard to cure liver fibrosis.