scholarly journals Cone Photoreceptors Develop Normally in the Absence of Functional Rod Photoreceptors in a Transgenic Swine Model of Retinitis Pigmentosa

2014 ◽  
Vol 55 (4) ◽  
pp. 2460 ◽  
Author(s):  
Juan P. Fernandez de Castro ◽  
Patrick A. Scott ◽  
James W. Fransen ◽  
James Demas ◽  
Paul J. DeMarco ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Swine Model ◽  
Cone Photoreceptors ◽  
Rod Photoreceptors ◽  
Transgenic Swine

scholarly journals A Pro23His Mutation Alters Prenatal Rod Photoreceptor Morphology in a Transgenic Swine Model of Retinitis Pigmentosa

2014 ◽  
Vol 55 (4) ◽  
pp. 2452 ◽  
Author(s):  
Patrick A. Scott ◽  
Juan P. Fernandez de Castro ◽  
Henry J. Kaplan ◽  
Maureen A. McCall
Keyword(s):  
Retinitis Pigmentosa ◽  
Swine Model ◽  
Rod Photoreceptor ◽  
Transgenic Swine

Photonic Crystal Light Collectors in Fish Retina Improve Vision in Turbid Water

Science ◽  
2012 ◽  
Vol 336 (6089) ◽  
pp. 1700-1703 ◽  
Author(s):  
Moritz Kreysing ◽  
Roland Pusch ◽  
Dorothee Haverkate ◽  
Meik Landsberger ◽  
Jacob Engelmann ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Photonic Crystal ◽  
Turbid Water ◽  
Functional Type ◽  
Cone Photoreceptors ◽  
Rod Photoreceptors ◽  
Spatial Noise ◽  
Cone Sensitivity ◽  
Gnathonemus Petersii ◽  
Fish Retina

Despite their diversity, vertebrate retinae are specialized to maximize either photon catch or visual acuity. Here, we describe a functional type that is optimized for neither purpose. In the retina of the elephantnose fish (Gnathonemus petersii), cone photoreceptors are grouped together within reflecting, photonic crystal–lined cups acting as macroreceptors, but rod photoreceptors are positioned behind these reflectors. This unusual arrangement matches rod and cone sensitivity for detecting color-mixed stimuli, whereas the photoreceptor grouping renders the fish insensitive to spatial noise; together, this enables more reliable flight reactions in the fish’s dim and turbid habitat as compared with fish lacking this retinal specialization.

scholarly journals Genetic rescue models refute nonautonomous rod cell death in retinitis pigmentosa

2017 ◽  
Vol 114 (20) ◽  
pp. 5259-5264 ◽  
Author(s):  
Susanne F. Koch ◽  
Jimmy K. Duong ◽  
Chun-Wei Hsu ◽  
Yi-Ting Tsai ◽  
Chyuan-Sheng Lin ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Retinitis Pigmentosa ◽  
High Efficiency ◽  
Genetic Rescue ◽  
Rod Photoreceptors ◽  
Rods And Cones ◽  
Promising Treatment ◽  
Low Efficiency ◽  
Rod Cell

Retinitis pigmentosa (RP) is an inherited neurodegenerative disease, in which the death of mutant rod photoreceptors leads secondarily to the non-cell autonomous death of cone photoreceptors. Gene therapy is a promising treatment strategy. Unfortunately, current methods of gene delivery treat only a fraction of diseased cells, yielding retinas that are a mosaic of treated and untreated rods, as well as cones. In this study, we created two RP mouse models to test whether dying, untreated rods negatively impact treated, rescued rods. In one model, treated and untreated rods were segregated. In the second model, treated and untreated rods were diffusely intermixed, and their ratio was controlled to achieve low-, medium-, or high-efficiency rescue. Analysis of these mosaic retinas demonstrated that rescued rods (and cones) survive, even when they are greatly outnumbered by dying photoreceptors. On the other hand, the rescued photoreceptors did exhibit long-term defects in their outer segments (OSs), which were less severe when more photoreceptors were treated. In summary, our study suggests that even low-efficiency gene therapy may achieve stable survival of rescued photoreceptors in RP patients, albeit with OS dysgenesis.

scholarly journals Genetic Reactivation of Cone Photoreceptors Restores Visual Responses in Retinitis Pigmentosa

Science ◽  
2010 ◽  
Vol 329 (5990) ◽  
pp. 413-417 ◽  
Author(s):  
V. Busskamp ◽  
J. Duebel ◽  
D. Balya ◽  
M. Fradot ◽  
T. J. Viney ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Cone Photoreceptors ◽  
Visual Responses

scholarly journals ℮-conome: an automated tissue counting platform of cone photoreceptors for rodent models of retinitis pigmentosa

2011 ◽  
Vol 11 (1) ◽  
Author(s):  
Emmanuelle Clérin ◽  
Nicolas Wicker ◽  
Saddek Mohand-Saïd ◽  
Olivier Poch ◽  
José-Alain Sahel ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Rodent Models ◽  
Cone Photoreceptors

Retinitis pigmentosa: cone photoreceptors starving to death

2009 ◽  
Vol 12 (1) ◽  
pp. 5-6 ◽  
Author(s):  
Paola Bovolenta ◽  
Elsa Cisneros
Keyword(s):  
Retinitis Pigmentosa ◽  
Cone Photoreceptors

scholarly journals AAV-Txnip prolongs cone survival and vision in mouse models of retinitis pigmentosa

2021 ◽  
Author(s):  
Yunlu Xue ◽  
Sean K. Wang ◽  
Parimal Rana ◽  
Emma R. West ◽  
Christin M. Hong ◽  
...  
Keyword(s):  
Retinitis Pigmentosa ◽  
Mouse Models ◽  
Retinal Disease ◽  
Disease Genes ◽  
Cone Photoreceptors ◽  
Adeno Associated Virus ◽  
Rescue Effect ◽  
Inherited Retinal Disease ◽  
High Acuity

AbstractRetinitis pigmentosa (RP) is an inherited retinal disease, affecting >20 million people worldwide. Loss of daylight vision typically occurs due to the dysfunction/loss of cone photoreceptors, the cell type that initiates our color and high acuity vision. Currently, there is no effective treatment for RP, other than gene therapy for a limited number of specific disease genes. To develop a gene-agnostic therapy, we screened ≈20 genes for their ability to prolong cone photoreceptor survival in vivo. Here, we report an adeno-associated virus (AAV) vector expressing Txnip, which prolongs the survival of cone photoreceptors and improves visual acuity in RP mouse models. A Txnip allele, C247S, which blocks the association of Txnip with thioredoxin, provides an even greater benefit. Additionally, the rescue effect of Txnip depends on lactate dehydrogenase b (Ldhb), and correlates with the presence of healthier mitochondria, suggesting that Txnip saves RP cones by enhancing their lactate catabolism.

scholarly journals Neuroinflammation as a Therapeutic Target in Retinitis Pigmentosa and Quercetin as Its Potential Modulator

Pharmaceutics ◽  
2021 ◽  
Vol 13 (11) ◽  
pp. 1935
Author(s):  
Joseph Thomas Ortega ◽  
Beata Jastrzebska
Keyword(s):  
Inflammatory Response ◽  
Retinitis Pigmentosa ◽  
Therapeutic Strategy ◽  
Treatment Strategies ◽  
Photoreceptor Cells ◽  
Rod Photoreceptors ◽  
Inflammatory Reactions ◽  
Environmental Light ◽  
The Stability ◽  
Visual Receptor

The retina is a multilayer neuronal tissue located in the back of the eye that transduces the environmental light into a neural impulse. Many eye diseases caused by endogenous or exogenous harm lead to retina degeneration with neuroinflammation being a major hallmark of these pathologies. One of the most prevalent retinopathies is retinitis pigmentosa (RP), a clinically and genetically heterogeneous hereditary disorder that causes a decline in vision and eventually blindness. Most RP cases are related to mutations in the rod visual receptor, rhodopsin. The mutant protein triggers inflammatory reactions resulting in the activation of microglia to clear degenerating photoreceptor cells. However, sustained insult caused by the abnormal genetic background exacerbates the inflammatory response and increases oxidative stress in the retina, leading to a decline in rod photoreceptors followed by cone photoreceptors. Thus, inhibition of inflammation in RP has received attention and has been explored as a potential therapeutic strategy. However, pharmacological modulation of the retinal inflammatory response in combination with rhodopsin small molecule chaperones would likely be a more advantageous therapeutic approach to combat RP. Flavonoids, which exhibit antioxidant and anti-inflammatory properties, and modulate the stability and folding of rod opsin, could be a valid option in developing treatment strategies against RP.

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