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Endoplasmic reticulum stress: New insights into the pathogenesis and treatment of retinal degenerative diseases.
Prog Retin Eye Res. 2020 Apr 06 [Online ahead of print]PR

Abstract

Physiological equilibrium in the retina depends on coordinated work between rod and cone photoreceptors and can be compromised by the expression of mutant proteins leading to inherited retinal degeneration (IRD). IRD is a diverse group of retinal dystrophies with multifaceted molecular mechanisms that are not fully understood. In this review, we focus on the contribution of chronically activated unfolded protein response (UPR) to inherited retinal pathogenesis, placing special emphasis on studies employing genetically modified animal models. As constitutively active UPR in degenerating retinas may activate pro-apoptotic programs associated with oxidative stress, pro-inflammatory signaling, dysfunctional autophagy, free cytosolic Ca2+ overload, and altered protein synthesis rate in the retina, we focus on the regulatory mechanisms of translational attenuation and approaches to overcoming translational attenuation in degenerating retinas. We also discuss current research on the role of the UPR mediator PERK and its downstream targets in degenerating retinas and highlight the therapeutic benefits of reprogramming PERK signaling in preclinical animal models of IRD. Finally, we describe pharmacological approaches targeting UPR in ocular diseases and consider their potential applications to IRD.

Authors+Show Affiliations

The University of Alabama at Birmingham, Department of Optometry and Vision Science, School of Optometry, USA. Electronic address: mgortk@uab.edu.The University of Alabama at Birmingham, Department of Optometry and Vision Science, School of Optometry, USA.The University of Alabama at Birmingham, Department of Optometry and Vision Science, School of Optometry, USA.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

32272207

Citation

Gorbatyuk, Marina S., et al. "Endoplasmic Reticulum Stress: New Insights Into the Pathogenesis and Treatment of Retinal Degenerative Diseases." Progress in Retinal and Eye Research, 2020, p. 100860.
Gorbatyuk MS, Starr CR, Gorbatyuk OS. Endoplasmic reticulum stress: New insights into the pathogenesis and treatment of retinal degenerative diseases. Prog Retin Eye Res. 2020.
Gorbatyuk, M. S., Starr, C. R., & Gorbatyuk, O. S. (2020). Endoplasmic reticulum stress: New insights into the pathogenesis and treatment of retinal degenerative diseases. Progress in Retinal and Eye Research, 100860. https://doi.org/10.1016/j.preteyeres.2020.100860
Gorbatyuk MS, Starr CR, Gorbatyuk OS. Endoplasmic Reticulum Stress: New Insights Into the Pathogenesis and Treatment of Retinal Degenerative Diseases. Prog Retin Eye Res. 2020 Apr 6;100860. PubMed PMID: 32272207.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Endoplasmic reticulum stress: New insights into the pathogenesis and treatment of retinal degenerative diseases. AU - Gorbatyuk,Marina S, AU - Starr,Christopher R, AU - Gorbatyuk,Oleg S, Y1 - 2020/04/06/ PY - 2019/12/22/received PY - 2020/03/08/revised PY - 2020/03/17/accepted PY - 2020/4/10/pubmed PY - 2020/4/10/medline PY - 2020/4/10/entrez KW - Neuroprotection KW - Retinal degeneration KW - Translation KW - Translational repressor KW - Unfolded protein response SP - 100860 EP - 100860 JF - Progress in retinal and eye research JO - Prog Retin Eye Res N2 - Physiological equilibrium in the retina depends on coordinated work between rod and cone photoreceptors and can be compromised by the expression of mutant proteins leading to inherited retinal degeneration (IRD). IRD is a diverse group of retinal dystrophies with multifaceted molecular mechanisms that are not fully understood. In this review, we focus on the contribution of chronically activated unfolded protein response (UPR) to inherited retinal pathogenesis, placing special emphasis on studies employing genetically modified animal models. As constitutively active UPR in degenerating retinas may activate pro-apoptotic programs associated with oxidative stress, pro-inflammatory signaling, dysfunctional autophagy, free cytosolic Ca2+ overload, and altered protein synthesis rate in the retina, we focus on the regulatory mechanisms of translational attenuation and approaches to overcoming translational attenuation in degenerating retinas. We also discuss current research on the role of the UPR mediator PERK and its downstream targets in degenerating retinas and highlight the therapeutic benefits of reprogramming PERK signaling in preclinical animal models of IRD. Finally, we describe pharmacological approaches targeting UPR in ocular diseases and consider their potential applications to IRD. SN - 1873-1635 UR - https://www.unboundmedicine.com/medline/citation/32272207/Endoplasmic_reticulum_stress:_New_insights_into_the_pathogenesis_and_treatment_of_retinal_degenerative_diseases L2 - https://linkinghub.elsevier.com/retrieve/pii/S1350-9462(20)30032-X DB - PRIME DP - Unbound Medicine ER -
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