Latent transforming growth factor beta binding protein 3 (LTBP3)

Latent transforming growth factor beta binding protein 3 (LTBP3) is an extracellular glycoprotein that regulates the bioavailability and activation of transforming growth factor beta (TGF-β) isoforms (TGFB1, TGFB2, TGFB3) by storing them in a latent state in the extracellular matrix[2]. LTBP3 forms a covalent complex with the TGF-β latency-associated peptide (LAP), facilitating the proper assembly, secretion, and tissue localization of latent TGF-β complexes[2]. Activation of TGF-β requires dissociation from LTBP3, allowing TGF-β to bind its receptors and initiate Smad-dependent and alternative (MAPK, non-Smad) signaling pathways that control diverse processes such as cell proliferation, differentiation, and tissue repair[1][2]. Mutations in LTBP3 disrupt these processes and cause rare inherited disorders such as geleophysic dysplasia 3, dental anomalies with short stature, and acromicric dysplasia[1][2]. LTBP3 is not itself a classic therapeutic target (such as a receptor or enzyme), but plays a critical regulatory role in major signaling pathways.

Other names

DASSGPHYSD3STHAG6pp6425latent TGF beta binding protein 3latent-transforming growth factor beta-binding protein 3

null (no direct drugs targeting LTBP3; LTBP3 modulates TGF-β activation, but clinically relevant drugs target TGF-β itself or its receptors, not LTBP3 directly)