ArticlePloS one2012
ER stress in retinal degeneration in S334ter Rho rats.
Article in PloS one, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 54 papers.
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Who cites it
54 citing papers in PubMed, 68 citations in OpenAlex.
- Axin1 stabilizes S-opsin and maintains cone photoreceptor survival by inhibiting GSK3β activity.Cell death discovery · 2026Article
- Synaptotagmin-7 drives stress-induced cardiomyocyte necroptosis via the p53-Bak-mPTP axis.Theranostics · 2026Article
- Gene Therapy for Rhodopsin Mutations.Cold Spring Harbor perspectives in medicine · 2022Article
- The cGMP system in normal and degenerating mouse neuroretina: New proteins with cGMP interaction potential identified by a proteomics approach.Journal of neurochemistry · 2021Article
- Deletion of M-Opsin Prevents M Cone Degeneration in a Mouse Model of Leber Congenital Amaurosis.The American journal of pathology · 2020Article
- Copper induce zebrafish retinal developmental defects via triggering stresses and apoptosis.Cell communication and signaling : CCS · 2020Article
- SRD005825 Acts as a Pharmacologic Chaperone of Opsin and Promotes Survival of Photoreceptors in an Animal Model of Autosomal Dominant Retinitis Pigmentosa.Translational vision science & technology · 2019Article
- ER stress and unfolded protein response in ocular health and disease.The FEBS journal · 2019Review
- Mutation in the intracellular chloride channel CLCC1 associated with autosomal recessive retinitis pigmentosa.PLoS genetics · 2018Article
- Phenotypic characterization of P23H and S334ter rhodopsin transgenic rat models of inherited retinal degeneration.Experimental eye research · 2018Article
- The role of the ER stress-response protein PERK in rhodopsin retinitis pigmentosa.Human molecular genetics · 2017Article
- Long-term photoreceptor rescue in two rodent models of retinitis pigmentosa by adeno-associated virus delivery of Stanniocalcin-1.Experimental eye research · 2017Article
- Calpain-5 gene expression in the mouse eye and brain.BMC research notes · 2017Article
- PERK and XBP1 differentially regulate CXCL10 and CCL2 production.Experimental eye research · 2017Article
- TNFa knockdown in the retina promotes cone survival in a mouse model of autosomal dominant retinitis pigmentosa.Biochimica et biophysica acta. Molecular basis of disease · 2017Article
- Loss of the cone-enrichedMolecular vision · 2017Article
- Insights from Genetic Model Systems of Retinal Degeneration: Role of Epsins in Retinal Angiogenesis and VEGFR2 Signaling.Journal of nature and science · 2017Article
- Structural and molecular bases of rod photoreceptor morphogenesis and disease.Progress in retinal and eye research · 2016Review
- Neuronatin is a stress-responsive protein of rod photoreceptors.Neuroscience · 2016Article
- Calpain-5 Expression in the Retina Localizes to Photoreceptor Synapses.Investigative ophthalmology & visual science · 2016Article
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Authors and funding
5 authors at 4 institutions in 1 country.
Funding
Abstract
The S334ter rhodopsin (Rho) rat (line 4) bears the rhodopsin gene with an early termination codon at residue 334 that is a model for several such mutations found in human patients with autosomal dominant retinitis pigmentosa (ADRP). The Unfolded Protein Response (UPR) is implicated in the pathophysiology of several retinal disorders including ADRP in P23H Rho rats. The aim of this study was to examine the onset of UPR gene expression in S334ter Rho retinas to determine if UPR is activated in ADRP animal models and to investigate how the activation of UPR molecules leads to the final demise of S334ter Rho photoreceptors. RT-PCR was performed to evaluate the gene expression profiles for the P10, P12, P15, and P21 stages of the development and progression of ADRP in S334ter Rho photoreceptors. We determined that during the P12-P15 period, ER stress-related genes are strongly upregulated in transgenic retinas, resulting in the activation of the UPR that was confirmed using western blot analysis and RT-PCR. The activation of UPR was associated with the increased expression of JNK, Bik, Bim, Bid, Noxa, and Puma genes and cleavage of caspase-12 that together with activated calpains presumably compromise the integrity of the mitochondrial MPTP, leading to the release of pro-apoptotic AIF1 into the cytosol of S334ter Rho photoreceptor cells. Therefore, two major cross-talking pathways, the UPR and mitochondrial MPTP occur in S334ter-4 Rho retina concomitantly and eventually promote the death of the photoreceptor cells.
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