ArticlePloS one2013
Endothelin-2-mediated protection of mutant photoreceptors in inherited photoreceptor degeneration.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 38 citations in OpenAlex.
- Dysfunction of Unc119, a Transducin-Binding Protein, Leads to Cone-Rod Dystrophy through Activating JAK-Stat and NF-κB Inflammatory Pathways in the Mouse Retina.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2025Article
- Functional significance of commonly regulated genes in mechanically and chemically induced retinal ganglion cell death in rat eyes.Scientific reports · 2025Article
- Combination of blockade of endothelin signalling and compensation of IGF1 expression protects the retina from degeneration.Cellular and molecular life sciences : CMLS · 2024Article
- Effects of different alkylating agents on photoreceptor degeneration and proliferative response of Müller glia.Scientific reports · 2024Article
- Retinitis Pigmentosa: Novel Therapeutic Targets and Drug Development.Pharmaceutics · 2023Review
- Article
- Early manifestations and differential gene expression associated with photoreceptor degeneration in Prom1-deficient retina.Disease models & mechanisms · 2021Article
- Oral Ursodeoxycholic Acid Crosses the Blood Retinal Barrier in Patients with Retinal Detachment and Protects Against Retinal Degeneration in an Ex Vivo Model.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2021Article
- Cd9 Protects Photoreceptors from Injury and Potentiates Edn2 Expression.Investigative ophthalmology & visual science · 2020Article
- A putative silencer variant in a spontaneous canine model of retinitis pigmentosa.PLoS genetics · 2020Article
- ADIPOR1 is essential for vision and its RPE expression is lost in the MfrpScientific reports · 2018Article
- p75Cell death & disease · 2017Article
- Endothelins Inhibit Osmotic Swelling of Rat Retinal Glial and Bipolar Cells by Activation of Growth Factor Signaling.Neurochemical research · 2016Article
- Article
- Animals deficient in C2Orf71, an autosomal recessive retinitis pigmentosa-associated locus, develop severe early-onset retinal degeneration.Human molecular genetics · 2015Article
- Generation and characterization of an endothelin-2 iCre mouse.Genesis (New York, N.Y. : 2000) · 2015Article
- Validation of endothelin B receptor antibodies reveals two distinct receptor-related bands on Western blot.Analytical biochemistry · 2015Article
- The Role of the Endothelin System in the Vascular Dysregulation Involved in Retinitis Pigmentosa.Journal of ophthalmology · 2015Review
- Endothelin2 Induces Expression of Genes Associated with Reactive Gliosis in Retinal Müller Cells.Current eye research · 2015Article
- STAT3 promotes survival of mutant photoreceptors in inherited photoreceptor degeneration models.Proceedings of the National Academy of Sciences of the United States of America · 2014Article
Corrections and comments
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Authors and funding
10 authors at 7 institutions in 2 countries.
Funding
Abstract
Expression of the Endothelin-2 (Edn2) mRNA is greatly increased in the photoreceptors (PRs) of mouse models of inherited PR degeneration (IPD). To examine the role of Edn2 in mutant PR survival, we generated Edn2(-/-) mice carrying homozygous Pde6b(rd1) alleles or the Tg(RHO P347S) transgene. In the Edn2(-/-) background, PR survival increased 110% in Pde6b(rd1/rd1) mice at post-natal (PN) day 15, and 60% in Tg(RHO P347S) mice at PN40. In contrast, PR survival was not increased in retinal explants of Pde6b(rd1/rd1) ; Edn2(-/-) mice. This finding, together with systemic abnormalities in Edn2(-/-) mice, suggested that the increased survival of mutant PRs in the Edn2(-/-) background resulted at least partly from the systemic EDN2 loss of function. To examine directly the role of EDN2 in mutant PRs, we used a scAAV5-Edn2 cDNA vector to restore Edn2 expression in Pde6b(rd1/rd1) ; Edn2(-/-) PRs and observed an 18% increase in PR survival at PN14. Importantly, PR survival was also increased after injection of scAAV5-Edn2 into Pde6b(rd1/rd1) retinas, by 31% at PN15. Together, these findings suggest that increased Edn2 expression is protective to mutant PRs. To begin to elucidate Edn2-mediated mechanisms that contribute to PR survival, we used microarray analysis and identified a cohort of 20 genes with >4-fold increased expression in Tg(RHO P347S) retinas, including Fgf2. Notably, increased expression of the FGF2 protein in Tg(RHO P347S) PRs was ablated in Tg(RHO P347S); Edn2(-/-) retinas. Our findings indicate that the increased expression of PR Edn2 increases PR survival, and suggest that the Edn2-dependent increase in PR expression of FGF2 may contribute to the augmented survival.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.