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Unfolded protein response

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Full-Text Articles in Medicine and Health Sciences

The Role Of Protein Synthesis Attenuation And Eif2-Alpha Phosphorylation In Retinal Degeneration, Christopher Ryan Starr Jan 2019

The Role Of Protein Synthesis Attenuation And Eif2-Alpha Phosphorylation In Retinal Degeneration, Christopher Ryan Starr

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Inherited retinal degeneration is a class of diseases in which there is no cure and very few treatment options available. An activated unfolded protein response has been detected in various animal models of retinal degenerative diseases. Several groups have found heightened activation of one pathway of the unfolded protein response in particular, the protein kinase R-like endoplasmic reticulum kinase signaling cascade, which converges on and phosphorylates the alpha subunit of eukaryotic translation initiation factor 2 to transiently halt protein synthesis and allow the cell to reestablish homeostasis. Although it is widely accepted that this signaling is inherently protective, hyperactivity or …


The Unfolded Protein Response And Calcium Dysregulation In Autosomal Dominant Retinitis Pigmentosa Animal Models., Vishal Madhukar Shinde Jan 2015

The Unfolded Protein Response And Calcium Dysregulation In Autosomal Dominant Retinitis Pigmentosa Animal Models., Vishal Madhukar Shinde

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The photoreceptor cell death in autosomal dominant retinitis pigmentosa (ADRP) is associated with molecular changes that occur due to mutations in a specific gene. S334ter RHO and P23H RHO are two ADRP rat models expressing mutant rhodopsin. The unfolded protein response (UPR) is a cellular stress response involved in the pathophysiology of several retinal disorders including P23H RHO rats. Considering our previous findings, we started our investigation by examining the status of UPR in S334ter RHO rats. As UPR tightly regulates several signaling pathways, we further studied whether UPR activation in S334ter and P23H RHO retina is accompanied by the …