Latent transforming growth factor beta-binding protein 4 (LTBP4)

Latent transforming growth factor beta-binding protein 4 (LTBP4) is a large secreted glycoprotein of the extracellular matrix, structurally related to fibrillins and characterized by multiple calcium-binding EGF-like repeat domains and TGFβ-binding cysteine-rich regions. LTBP4 covalently binds latent TGFβ1 complexes, mediating their incorporation and regulated activation within the matrix, and thereby controls multiple aspects of TGFβ signaling including development, immunity, wound healing, fibrosis, and cancer progression. LTBP4 also plays a non-TGFβ-dependent role in elastic fiber assembly (elastogenesis) via interactions with matrix proteins such as fibulin-4, fibulin-5, and fibrillin-1. Mutations in LTBP4 cause autosomal recessive cutis laxa type 1C, while altered expression is implicated in several acquired diseases including scleroderma, certain cancers, and fibrotic conditions. Although not a classic "drug target" receptor or enzyme, LTBP4 is emerging as a candidate biomarker and possible therapeutic modulator in ECM-related diseases and TGFβ pathway disorders.

Other names

Latent-transforming growth factor beta-binding protein 4LTBP4LTBP-4LTBP4LFLJ46318FLJ90018ARCL1CLTBP4Slatent-transforming growth factor beta-binding protein 4L

For theoretical or investigational drugs: Modulation of TGFβ signaling via interfering with LTBP4/TGFβ complex formation or activation. Therapeutic restoration/promotion of elastogenesis by influencing LTBP4 function (preclinical).