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Fuchs endothelial corneal dystrophy: The vicious cycle of Fuchs pathogenesis.
Prog Retin Eye Res. 2020 May 08 [Online ahead of print]PR

Abstract

Fuchs endothelial corneal dystrophy (FECD) is the most common primary corneal endothelial dystrophy and the leading indication for corneal transplantation worldwide. FECD is characterized by the progressive decline of corneal endothelial cells (CECs) and the formation of extracellular matrix (ECM) excrescences in Descemet's membrane (DM), called guttae, that lead to corneal edema and loss of vision. FECD typically manifests in the fifth decades of life and has a greater incidence in women. FECD is a complex and heterogeneous genetic disease where interaction between genetic and environmental factors results in cellular apoptosis and aberrant ECM deposition. In this review, we will discuss a complex interplay of genetic, epigenetic, and exogenous factors in inciting oxidative stress, auto(mito)phagy, unfolded protein response, and mitochondrial dysfunction during CEC degeneration. Specifically, we explore the factors that influence cellular fate to undergo apoptosis, senescence, and endothelial-to-mesenchymal transition. These findings will highlight the importance of abnormal CEC-DM interactions in triggering the vicious cycle of FECD pathogenesis. We will also review clinical characteristics, diagnostic tools, and current medical and surgical management options for FECD patients. These new paradigms in FECD pathogenesis present an opportunity to develop novel therapeutics for the treatment of FECD.

Authors+Show Affiliations

Cornea Center of Excellence, Schepens Eye Research Institute, Harvard Medical School, Boston, MA, United States; Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, United States; Department of Ophthalmology, Harvard Medical School, Boston, MA, United States.Cornea Center of Excellence, Schepens Eye Research Institute, Harvard Medical School, Boston, MA, United States; Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, United States; Department of Ophthalmology, Harvard Medical School, Boston, MA, United States.Cornea Center of Excellence, Schepens Eye Research Institute, Harvard Medical School, Boston, MA, United States; Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, United States; Department of Ophthalmology, Harvard Medical School, Boston, MA, United States.Cornea Center of Excellence, Schepens Eye Research Institute, Harvard Medical School, Boston, MA, United States; Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, United States; Department of Ophthalmology, Harvard Medical School, Boston, MA, United States.Cornea Center of Excellence, Schepens Eye Research Institute, Harvard Medical School, Boston, MA, United States; Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, United States; Department of Ophthalmology, Harvard Medical School, Boston, MA, United States.Cornea Center of Excellence, Schepens Eye Research Institute, Harvard Medical School, Boston, MA, United States; Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, United States; Department of Ophthalmology, Harvard Medical School, Boston, MA, United States. Electronic address: ula_jurkunas@meei.harvard.edu.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32438095

Citation

Ong Tone, Stephan, et al. "Fuchs Endothelial Corneal Dystrophy: the Vicious Cycle of Fuchs Pathogenesis." Progress in Retinal and Eye Research, 2020, p. 100863.
Ong Tone S, Kocaba V, Böhm M, et al. Fuchs endothelial corneal dystrophy: The vicious cycle of Fuchs pathogenesis. Prog Retin Eye Res. 2020.
Ong Tone, S., Kocaba, V., Böhm, M., Wylegala, A., White, T. L., & Jurkunas, U. V. (2020). Fuchs endothelial corneal dystrophy: The vicious cycle of Fuchs pathogenesis. Progress in Retinal and Eye Research, 100863. https://doi.org/10.1016/j.preteyeres.2020.100863
Ong Tone S, et al. Fuchs Endothelial Corneal Dystrophy: the Vicious Cycle of Fuchs Pathogenesis. Prog Retin Eye Res. 2020 May 8;100863. PubMed PMID: 32438095.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Fuchs endothelial corneal dystrophy: The vicious cycle of Fuchs pathogenesis. AU - Ong Tone,Stephan, AU - Kocaba,Viridiana, AU - Böhm,Myriam, AU - Wylegala,Adam, AU - White,Tomas L, AU - Jurkunas,Ula V, Y1 - 2020/05/08/ PY - 2019/11/13/received PY - 2020/04/05/revised PY - 2020/04/10/accepted PY - 2020/5/22/pubmed PY - 2020/5/22/medline PY - 2020/5/22/entrez KW - Apoptosis KW - Corneal endothelium KW - Fuchs endothelial corneal dystrophy KW - Guttae KW - Mitochondria KW - Oxidative stress SP - 100863 EP - 100863 JF - Progress in retinal and eye research JO - Prog Retin Eye Res N2 - Fuchs endothelial corneal dystrophy (FECD) is the most common primary corneal endothelial dystrophy and the leading indication for corneal transplantation worldwide. FECD is characterized by the progressive decline of corneal endothelial cells (CECs) and the formation of extracellular matrix (ECM) excrescences in Descemet's membrane (DM), called guttae, that lead to corneal edema and loss of vision. FECD typically manifests in the fifth decades of life and has a greater incidence in women. FECD is a complex and heterogeneous genetic disease where interaction between genetic and environmental factors results in cellular apoptosis and aberrant ECM deposition. In this review, we will discuss a complex interplay of genetic, epigenetic, and exogenous factors in inciting oxidative stress, auto(mito)phagy, unfolded protein response, and mitochondrial dysfunction during CEC degeneration. Specifically, we explore the factors that influence cellular fate to undergo apoptosis, senescence, and endothelial-to-mesenchymal transition. These findings will highlight the importance of abnormal CEC-DM interactions in triggering the vicious cycle of FECD pathogenesis. We will also review clinical characteristics, diagnostic tools, and current medical and surgical management options for FECD patients. These new paradigms in FECD pathogenesis present an opportunity to develop novel therapeutics for the treatment of FECD. SN - 1873-1635 UR - https://www.unboundmedicine.com/medline/citation/32438095/Fuchs_endothelial_corneal_dystrophy:_The_vicious_cycle_of_Fuchs_pathogenesis L2 - https://linkinghub.elsevier.com/retrieve/pii/S1350-9462(20)30035-5 DB - PRIME DP - Unbound Medicine ER -
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