Retinitis pigmentosa (RP) is an inherited ocular disorder with no effective treatment. RP onset and progression trigger a cascade of retinal disorders that lead to the death of photoreceptors. After photoreceptors death, neuronal, glial and vascular remodeling can be observed in the retina. The purpose of this study was to study if thioredoxin (TRX) administration is able to decrease photoreceptor death in an animal model of RP (rd1 mouse), but also if it is able to modulate the retinal oxidative stress, glial and vascular changes that can be observed as the disease progresses. Wild type and rd1 mice received several doses of TRX. After treatment, animals were euthanized at postnatals days 11, 17, or 28. Glutathione (GSH) and other thiol compounds were determined by high performance liquid chromatography (HPLC). Glial fibrilary acidic protein (GFAP) and anti-ionized calcium binding adaptor molecule 1 (Iba1) were studied by immunohistochemistry. Vascular endothelial growth factor (VEGF) and hepatic growth factor (HGF) expression were determined by western blot. TRX administration significantly diminished cell death in rd1 mouse retinas and increased GSH retinal concentrations at postnatal day 11 (PN11). TRX was also able to reverse glial alterations at PN11 and PN17. No alterations were observed in retinal VEGF and HGF expression in rd1 mice. In conclusion, TRX treatment decreases photoreceptor death in the first stages of RP and this protective effect may be due in part to the GSH system activation and to a partially decrease in inflammation.
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http://dx.doi.org/10.3389/fphar.2020.590572 | DOI Listing |
Vision (Basel)
January 2025
Sztárai Institute, University of Tokaj, 3950 Sárospatak, Hungary.
Retinitis pigmentosa (RP) encompasses inherited retinal dystrophies, appearing either as an isolated eye condition or as part of a broader systemic syndrome, known as syndromic RP. In these cases, RP includes systemic symptoms impacting other organs, complicating diagnosis and management. This review highlights key systemic syndromes linked with RP, such as Usher, Bardet-Biedl, and Alström syndromes, focusing on genetic mutations, inheritance, and clinical symptoms.
View Article and Find Full Text PDFCurr Opin Biotechnol
January 2025
Shanghai Frontiers Science Center of Genome Editing and Cell Therapy, Biomedical Synthetic Biology Research Center, Shanghai Key Laboratory of Regulatory Biology, Institute of Biomedical Sciences and School of Life Sciences, East China Normal University, Shanghai 200241, China; Wuhu Hospital, Health Science Center, East China Normal University, Wuhu 241001, China; Shanghai Academy of Natural Sciences, East China Normal University, Shanghai, China. Electronic address:
Optogenetics, an innovative approach integrating photonics and genetic engineering, enables precise control over molecular and cellular processes, opening up exciting new opportunities for precision-guided medicine. In this review, we highlight recent advances in optogenetic tools and their applications across a range of medical conditions, including vision restoration in retinitis pigmentosa via light-activated ion channels, precise immune response modulation in cancer immunotherapy, and blood glucose management in diabetes through controllable drug release. Optogenetics also plays a critical role in bioelectronic medicine, enabling seamless communication between electronic systems and biological tissues to enhance therapeutic precision.
View Article and Find Full Text PDFRetina
January 2025
Shiley Eye Institute, University of California, San Diego, CA, USA.
Purpose: To characterize retinal vessel whitening (RVW) in Retinitis Pigmentosa (RP).
Methods: Single-center cross-sectional study. Review of clinical notes of clinically confirmed RP patients was performed followed by grading ultra-widefield imaging.
J Psychiatr Pract
January 2025
Department of Psychiatry, Centre for Addiction Medicine, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
Charles Bonnet syndrome (CBS) is a clinical condition in which patients with visual impairment experience visual hallucinations (VH) in the presence of clear consciousness. It typically occurs in elderly people and confuses clinicians with multiple differential diagnoses due to VH, which can be present in a variety of clinical conditions ranging from psychosis to neurocognitive disorders (eg, neurocognitive disorder with Lewy bodies). In the latter, the concomitant presence of cognitive decline and parkinsonism aids the diagnosis.
View Article and Find Full Text PDFMol Neurodegener
January 2025
The Jackson Laboratory, Bar Harbor, ME, 04609, USA.
Background: Age is the principal risk factor for neurodegeneration in both the retina and brain. The retina and brain share many biological properties; thus, insights into retinal aging and degeneration may shed light onto similar processes in the brain. Genetic makeup strongly influences susceptibility to age-related retinal disease.
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