The S334ter-3 rat is a transgenic model of retinal degeneration (RD) developed to express a rhodopsin mutation similar to that found in human retinitis pigmentosa. Due to this advantage over other models of RD, a few retina transplant studies have been reported on this animal model. Currently, no information is available on cone photoreceptor changes that occur in the S334ter RD model. In this study, we investigated the effect of RD on the morphology, distribution, and synaptic connectivity of short-wavelength cones (S-cones) during development of S334ter-3 rat retinas. At P21 RD retinas, the outer-nuclear layer was significantly narrower, while S-cones showed shortening of their segments and axons compared to control retinas. From P90 onward, S-opsin-immunoreactive cells appeared at the outer margin of the inner-nuclear layer of RD retinas. Double-labelling experiments showed these cells contained recoverin and cone arrestin. Furthermore, ultra-structure study showed that synaptic ribbons are conserved in the S-cone at P180 RD retinas. Although cell density of S-cones significantly dropped after P90, survival rates depended on the retinal region. Overall, the S334ter-3 RD model shows hallmarks of cone remodelling due to photoreceptor degeneration.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2855539 | PMC |
| http://dx.doi.org/10.1016/j.brainres.2010.01.051 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
represses short-wavelength cone genes to preserve long-wavelength cone and rod photoreceptor identity.
Proc Natl Acad Sci U S A
November 2024
Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO 63110.
The role of transcription factors in photoreceptor gene regulation is fairly well understood, but knowledge of the cell-type-specific function of transcriptional cofactors remains incomplete. Here, we show that the transcriptional corepressor promotes rod differentiation and represses short-wavelength cone genes in long-wavelength cones in zebrafish. In retinas, red cones are transformed into hybrid red/ultraviolet (UV) cones, green cones are absent, the number of blue cones is approximately doubled, and the number of rods is greatly reduced.
View Article and Find Full Text PDFEvaluation of Retinal Structure and Visual Function in Blue Cone Monochromacy to Develop Clinical Endpoints for L-opsin Gene Therapy.
Int J Mol Sci
October 2024
Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.
Article Synopsis
- The study explores gene therapy targeting L-cone opsin expression as a treatment for blue cone monochromacy (BCM), a condition caused by the lack of long- and middle-wavelength-sensitive cone function.
- Eight patients with BCM underwent various assessments, including optical coherence tomography and chromatic perimetry, revealing outer retinal changes and significant deficiencies in color vision and visual acuity.
- The research emphasizes the need for specialized outcome measures, such as dark-adapted microperimetry and specific visual acuity tests, to evaluate the efficacy and safety of potential future treatments in clinical trials.
Parallel Spectral Tuning of a Cone Visual Pigment Provides Evidence for Ancient Deep-Sea Adaptations in Cetaceans.
Genome Biol Evol
October 2024
College of Life Sciences, Shaanxi Normal University, Xi'an, Shaanxi, China.
Article Synopsis
- Dichromatic color vision in many mammals is based on two cone visual pigments, but early cetaceans lost their violet-sensitive pigment, resulting in monochromatic vision.
- Research shows that while some cetaceans have intact genes for middle/long wavelength-sensitive pigments, they lack spectral sensitivity, indicating a loss of cone-mediated vision.
- Ancestral analysis suggests that the M/LWS pigments of baleen and sperm whale ancestors evolved to be more sensitive to shorter wavelengths, likely as adaptations for deep-sea living, despite further losses in their function.
Adaptive optics scanning laser ophthalmoscopy in a heterogenous cohort with Stargardt disease.
Sci Rep
October 2024
Department of Experimental Psychology, University of Oxford, Oxford, OX2 6GG, UK.
Image based cell-specific biomarkers will play an important role in monitoring treatment outcomes of novel therapies in patients with Stargardt (STGD1) disease and may provide information on the exact mechanism of retinal degeneration. This study reports retinal image features from conventional clinical imaging and from corresponding high-resolution imaging with a confocal adaptive optics scanning laser ophthalmoscope (AOSLO) in a heterogenous cohort of patients with Stargardt (STGD1) disease. This is a prospective observational study in which 16 participants with clinically and molecularly confirmed STGD1, and 7 healthy controls underwent clinical assessment and confocal AOSLO imaging.
View Article and Find Full Text PDFRestoration of cone-circuit functionality in the regenerating adult zebrafish retina.
Dev Cell
August 2024
CRTD - Center for Regenerative Therapies TU Dresden, CMCB, Technische Universität Dresden, Fetscherstraße 105, 01307 Dresden, Germany; CMCB - Center for Molecular and Cellular Bioengineering, Technische Universität Dresden, Tatzberg 47-51, 01307 Dresden, Germany; PoL - Excellence Cluster Physics of Life, TU Dresden, Fetscherstrasse 105, 01307 Dresden, Germany. Electronic address:
Unlike humans, teleosts like zebrafish exhibit robust retinal regeneration after injury from endogenous stem cells. However, it is unclear if regenerating cone photoreceptors regain physiological function and integrate correctly into post-synaptic circuits. We used two-photon calcium imaging of living adult retina to examine photoreceptor responses before and after light-induced lesions.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!