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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 …