Latent transforming growth factor binding protein-2 (LTBP2), an IPF biomarker of clinical decline, promotes TGF-beta signaling and lung fibrosis in mice.

ABSTRACT: The identification of clinically predictive serum biomarkers for pulmonary fibrosis is a significant challenge and important goal. Multiple recent proteomic biomarker studies have identified latent transforming growth factor binding protein-2 (LTBP2) as a circulating factor associated with disease progression in fibrotic lung diseases in humans (including IPF), but its role in the development of fibrosis is incompletely defined. LTBP2 competes with the large latent transforming growth factor-beta (TGFβ) complex (LLC) for binding to the N-terminus of fibrillin and is thought to promote the release of active TGFβ. We hypothesized that LTBP2 deficiency would promote LLC sequestration in matrix and reduce TGFβ signaling. We recently reported an LTBP2 knockout (Ltbp2 ^-/-) mous

SUBMITTER: Bodmer NK 

PROVIDER: S-EPMC12247642 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature