Activation of TGF-β signaling induces cell death via the unfolded protein response in Fuchs endothelial corneal dystrophy.

ABSTRACT: Fuchs endothelial corneal dystrophy (FECD) is a slowly progressive bilateral disease of corneal endothelium in which accumulation of extracellular matrix (ECM) and loss of corneal endothelial cells (CECs) are phenotypic features. The corneal endothelium maintains corneal transparency by regulating water hydration; consequently, corneal endothelial dysfunction causes serious vision loss. The only therapy for corneal haziness due to corneal endothelial diseases, including FECD, is corneal transplantation using donor corneas, and no pharmaceutical treatment is available. We provide evidence that the expression levels of transforming growth factor-β (TGF-β) isoforms and TGF-β receptors are high in the corneal endothelium of patients with FECD. A cell model based on patients with FECD shows tha

SUBMITTER: Okumura N 

PROVIDER: S-EPMC5533742 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature