Sustained Activation of the Unfolded Protein Response Induces Cell Death in Fuchs' Endothelial Corneal Dystrophy

• Published in: Investigative ophthalmology & visual science Vol. 58; no. 9; p. 3697
• Main Authors: Okumura, Naoki, Kitahara, Miu, Okuda, Hirokazu, Hashimoto, Keisuke, Ueda, Emi, Nakahara, Makiko, Kinoshita, Shigeru, Young, Robert D., Quantock, Andrew J., Tourtas, Theofilos, Schlötzer-Schrehardt, Ursula, Kruse, Friedrich, Koizumi, Noriko
• Format: Journal Article
• Language: English
• Published: United States 01.07.2017
• Subjects: Agrin - metabolism; Apoptosis; Cells, Cultured; Collagen Type I - metabolism; Descemet Membrane - metabolism; Descemet Membrane - pathology; Endoplasmic Reticulum - metabolism; Endoplasmic Reticulum - pathology; Endothelium, Corneal - metabolism; Extracellular Matrix Proteins - metabolism; Fibronectins - metabolism; Fuchs' Endothelial Dystrophy - metabolism; Fuchs' Endothelial Dystrophy - pathology; Heat-Shock Proteins - metabolism; Humans; Immunohistochemistry; Membrane Potential, Mitochondrial - physiology; Middle Aged; Oxidative Stress; Protein Aggregation, Pathological - metabolism; Protein Aggregation, Pathological - pathology; Real-Time Polymerase Chain Reaction; Unfolded Protein Response - physiology

The unfolded protein response (UPR) is believed to play a role in the pathogenesis of Fuchs' endothelial corneal dystrophy (FECD). The purpose of this study was to investigate whether unfolded proteins accumulate in the corneal endothelium in FECD and if they are involved in triggering cell dea...