Differential Dimerization of Variants Linked to Enhanced S-Cone Sensitivity Syndrome (ESCS) Located in the NR2E3 Ligand-Binding Domain
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The vertebrate retina is a model system of the development of the central nervous system. Because of its rapid development and the availability of many molecular tools the zebrafish is an established model of retinal development. Development and maintenanc ...
RPE65-related Leber's congenital amaurosis (LCA) is a rod-cone dystrophy whose clinical outcome is mainly attributed to the loss of rod photoreceptors followed by cone degeneration. Pathogenesis in Rpe65-/- mice is characterized by a slow and progressive d ...
Sight restoration after retinal degeneration still represents an unmet goal. Since therapeutic strategies increasingly require interdisciplinary competencies, photoreceptor degeneration is attracting several research areas, including: life science, materia ...
Purpose: To analyze in vivo the function of chicken acidic leucine-rich epidermal growth factor-like domain containing brain protein/Neuroglycan C (gene symbol: Cspg5) during retinal degeneration in the Rpe65(-/-) mouse model of Leber congenital amaurosis. ...
Progressive degeneration of photoreceptors is one of the major causes of adult blindness in “industrialized” countries; as an example, Retinitis pigmentosa defines a set of monogenic hereditary retinal diseases, with a prevalence of 1:4000 worldwide, cause ...
Purpose: Sight restoration is one of the new frontiers for prosthetic devices that enable the electrical stimulation of neurons. In particular, diseases that affect the retinal pigment epithelium and photoreceptors but preserve the inner retinal layers are ...
Organic semiconductors have emerged in the past two decades as promising materials for many technological applications. Thanks to their unique optoelectronic properties, they represent an ideal system to mimic natural photoreceptor functioning. This simila ...
The retina is one of the best known quantum detectors with rods able to reliably respond to single photons. However, estimates on the number of photons eliciting conscious perception, based on signal detection theory, are systematically above these values ...
Replacement strategies arise as promising approaches in case of inherited retinal dystrophies leading to blindness. A fully organic retinal prosthesis made of conjugated polymers layered onto a silk fibroin substrate is engineered. First, the biophysical a ...
The 'red-green' pathway of the retina is classically recognized as one of the retinal mechanisms allowing humans to gather color information from light, by combining information from L-cones and M-cones in an opponent way. The precise retinal circuitry tha ...