The endoplasmic reticulum: Homeostasis and crosstalk in retinal health and disease
The endoplasmic reticulum (ER) is the largest intracellular organelle carrying out a broad
range of important cellular functions including protein biosynthesis, folding, and trafficking …
range of important cellular functions including protein biosynthesis, folding, and trafficking …
[HTML][HTML] Mechanisms of Rhodopsin-Related Inherited Retinal Degeneration and Pharmacological Treatment Strategies
M Azam, B Jastrzebska - Cells, 2025 - mdpi.com
Retinitis pigmentosa (RP) is a hereditary disease characterized by progressive vision loss
ultimately leading to blindness. This condition is initiated by mutations in genes expressed in …
ultimately leading to blindness. This condition is initiated by mutations in genes expressed in …
Aggregation of rhodopsin mutants in mouse models of autosomal dominant retinitis pigmentosa
S Vasudevan, S Senapati, M Pendergast… - Nature …, 2024 - nature.com
Mutations in rhodopsin can cause it to misfold and lead to retinal degeneration. A
distinguishing feature of these mutants in vitro is that they mislocalize and aggregate. It is …
distinguishing feature of these mutants in vitro is that they mislocalize and aggregate. It is …
Compensation of inner retina to early-stage photoreceptor degeneration in a RhoP23H/+ mouse model of retinitis pigmentosa
Retinitis pigmentosa (RP) is an inherited retinal disorder characterized by the degeneration
of photoreceptors. Rho P23H/+ mice, which carry a Pro23His mutation in the RHODOPSIN …
of photoreceptors. Rho P23H/+ mice, which carry a Pro23His mutation in the RHODOPSIN …
SON-dependent nuclear speckle rejuvenation alleviates proteinopathies
Current treatments targeting individual protein quality control have limited efficacy in
alleviating proteinopathies, highlighting the prerequisite for a common upstream druggable …
alleviating proteinopathies, highlighting the prerequisite for a common upstream druggable …
Drug discovery strategies for inherited retinal degenerations
A Das, Y Imanishi - Biology, 2022 - mdpi.com
Simple Summary Inherited retinal degeneration is a group of heterogeneous genetic
disorders impairing vision. In these diseases, photoreceptor or retinal ganglion cells become …
disorders impairing vision. In these diseases, photoreceptor or retinal ganglion cells become …
Morphological characterization of retinal development from birth to adulthood via retinal thickness assessment in mice: a systematic review
The morphology and thickness of the retinal layers are valuable biomarkers for retinal health
and development. The retinal layers in mice are similar to those in humans; thus, a mouse is …
and development. The retinal layers in mice are similar to those in humans; thus, a mouse is …
Identification of small molecular chaperones binding p23h mutant opsin through an in silico structure-based approach
F Picarazzi, M Zuanon, G Pasqualetto… - Journal of Chemical …, 2022 - ACS Publications
N-terminal P23H opsin mutation accounts for most of retinitis pigmentosa (RP) cases. P23H
functions and folding can be rescued by small chaperone ligands, which contributes to …
functions and folding can be rescued by small chaperone ligands, which contributes to …
[HTML][HTML] Oral 8-aminoguanine against age-related retinal degeneration
Visual decline in the elderly is often attributed to retinal aging, which predisposes the tissue
to pathologies such as age-related macular degeneration. Currently, effective oral …
to pathologies such as age-related macular degeneration. Currently, effective oral …
Sex-specific attenuation of photoreceptor degeneration by reserpine in a rhodopsin P23H rat model of autosomal dominant retinitis pigmentosa
Inherited retinal degenerations (IRDs) constitute a group of clinically and genetically diverse
vision-impairing disorders. Retinitis pigmentosa (RP), the most common form of IRD, is …
vision-impairing disorders. Retinitis pigmentosa (RP), the most common form of IRD, is …