Endoplasmic reticulum stress: New insights into the pathogenesis and treatment of retinal degenerative diseases.

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

SUBMITTER: Gorbatyuk MS 

PROVIDER: S-EPMC7541398 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature