Latent transforming growth factor beta-binding protein 2 (LTBP2)

Latent transforming growth factor beta-binding protein 2 (LTBP2) is a large extracellular matrix protein and a member of the LTBP family, which shares significant structural similarity with fibrillins[2][3][6]. Unlike other LTBPs, LTBP2 does not bind covalently with latent TGF-beta complexes, but is widely associated with fibrillin-1-containing microfibrils and is thought to play an integral structural role in the organization and assembly of elastic fibers and the extracellular matrix[1][2][6]. LTBP2 specifically binds the amino-terminal region of fibrillin-1, potentially competing for binding sites used by LTBP1, and is suggested to act as a negative modulator of latent TGF-beta storage on microfibrils[1]. Mutations in LTBP2 have been associated with inherited connective tissue diseases such as Weill-Marchesani syndrome 3 and congenital glaucoma[2][3][5]. There are no approved drugs or known therapeutic interventions targeting LTBP2, and it is not currently considered a therapeutic drug target in itself.

Other names

C14orf141GLC3DLTBP3MSPKAMSTP031WMS3LTBP-2latent transforming growth factor beta binding protein 2