Article
Anatomy & Morphology
Debora Napoli, Enrica Strettoi
Summary: Retinal pigment epithelium (RPE) is a specialized cell layer dedicated to supporting and protecting the retina. In retinitis pigmentosa (RP), degeneration of the photoreceptors can lead to abnormal organization and compromised barrier function of RPE cells. The biological mechanisms behind these changes are largely unknown. A study using a mouse model of RP suggests that inflammatory responses may be involved in remodeling of RPE, and further investigation is needed.
JOURNAL OF ANATOMY
(2023)
Article
Multidisciplinary Sciences
Divya Ail, Diane Nava, In Pyo Hwang, Elena Brazhnikova, Celine Nouvel-Jaillard, Alexandre Dentel, Corentin Joffrois, Lionel Rousseau, Julie Degardin, Stephane Bertin, Jose-Alain Sahel, Olivier Goureau, Serge Picaud, Deniz Dalkara
Summary: To test novel therapies, suitable nonhuman primate models are needed, but there is a lack of relevant macaque models of retinal degeneration. Researchers have generated three distinct inducible cynomolgus macaque models of retinal degeneration to address this issue. Among the three models, the CRISPR-Cas9-based approach is the most advantageous for recapitulating disease-specific features and ease of implementation. The acute model, on the other hand, degenerates the fastest and is the most relevant for testing end-stage vision restoration therapies.
Article
Ophthalmology
Xiaozhen Liu, Ruixuan Jia, Xiang Meng, Ying Li, Liping Yang
Summary: Studies on treating adRP using CRISPR/Cas9 targeting RHO show potential therapeutic effects, but the development of humanized mouse models is still in its early stages.
EXPERIMENTAL EYE RESEARCH
(2022)
Article
Multidisciplinary Sciences
Teemu Turunen, Ari Koskelainen
Summary: The study reveals that phosphodiesterase-6 (PDE6) is regulated by calcium, playing a crucial role in converting photon information to bioelectrical signals in vertebrate photoreceptor cells. By decreasing rod intracellular Ca2+ concentration, the flash response recovery of photoreceptors can be accelerated, broadening their operating range and functionality.
SCIENTIFIC REPORTS
(2021)
Article
Biochemistry & Molecular Biology
Miriam Ryl, Alexander Urbasik, Kaspar Gierke, Norbert Babai, Anneka Joachimsthaler, Andreas Feigenspan, Renato Frischknecht, Nina Stallwitz, Anna Fejtova, Jan Kremers, Julia von Wittgenstein, Johann Helmut Brandstaetter
Summary: The study investigated the impact of mutations in the Bassoon protein on the synaptic function and structure of photoreceptor cells in mice. Results showed that the synaptic defects were more severe in Bsn(gt/gt) mice, while expression of the Bsn(gt) allele led to cone photoreceptor death and neurite sprouting in the outer retina.
Article
Multidisciplinary Sciences
Miguel Vizoso, Colin E. J. Pritchard, Lorenzo Bombardelli, Bram van den Broek, Paul Krimpenfort, Roderick L. Beijersbergen, Kees Jalink, Jacco van Rheenen
Summary: The authors report an optimised photoactivatable Cre recombinase system, doxycycline- and light-inducible Cre recombinase (DiLiCre), and generate a DiLiCre mouse line which they use for mutagenesis in vivo and positional cell-tracing.
NATURE COMMUNICATIONS
(2022)
Article
Cell Biology
Wanjing Huang, Qiang Xu, Jing Su, Lei Tang, Zhao-zhe Hao, Chuan Xu, Ruifeng Liu, Yuhui Shen, Xuan Sang, Nana Xu, Xiaoxiu Tie, Zhichao Miao, Xialin Liu, Ying Xu, Feng Liu, Yizhi Liu, Sheng Liu
Summary: In this study, we characterized the transcriptomic, morphological, and functional features of 472 high-quality RGCs using Patch-seq. The findings provide functional and morphological annotation for different transcriptomic-defined cell types in a previously established RGC atlas. The research highlights the convergence of different modalities in defining RGC identity and identifies differentially expressed genes among RGC subtypes, which could serve as candidate marker genes for functional studies.
Article
Medicine, Research & Experimental
Elliot H. Choi, Susie Suh, David E. Einstein, Henri Leinonen, Zhiqian Dong, Sriganesh Ramachandra Rao, Steven J. Fliesler, Seth Blackshaw, Minzhong Yu, Neal S. Peachey, Krzysztof Palczewski, Philip D. Kiser
Summary: This study presents a novel RPE-specific knockin mouse line with accurate introduction of the CreER(T2) gene into retinal pigment epithelial cells, demonstrating high efficiency and absolute RPE-specific recombination ability for studying gene function and pathophysiology in the RPE.
Article
Multidisciplinary Sciences
Jen-Zen Chuang, Nan Yang, Nobuyuki Nakajima, Wataru Otsu, Cheng Fu, Howard Hua Yang, Maxwell Ping Lee, Armaan Fazal Akbar, Tudor Constantin Badea, Ziqi Guo, Afnan Nuruzzaman, Kuo-Shun Hsu, Joshua L. Dunaief, Ching-Hwa Sung
Summary: In this study, researchers observed that mice lacking chloride intracellular channel 4 in retinal pigment epithelium exhibited a full spectrum of functional and pathological features of dry AMD, including drusen formation. Multidisciplinary longitudinal studies revealed a mechanistic link between RPE cell-autonomous aberrant lipid metabolism and transport and drusen formation.
NATURE COMMUNICATIONS
(2022)
Article
Biology
Guanping Feng, Aby Joseph, Kosha Dholakia, Fei Shang, Charles W. Pfeifer, Derek Power, Krishnan Padmanabhan, Jesse Schallek
Summary: In recent years, it has been possible to image single neurons, glia, and vascular cells in vivo to study local networks of activity in the living brain. However, a similar method has not yet been developed for the constantly moving eye. In this study, a novel head-fixed preparation was characterized to enable high-resolution retinal imaging at the single-cell level in awake mice. The study revealed new functional attributes of the eye that are overlooked by anesthesia and highlighted the benefits of studying retinal physiology without anesthesia.
COMMUNICATIONS BIOLOGY
(2023)
Article
Multidisciplinary Sciences
Joaquin Araya-Arriagada, Sebastian Garay, Cristobal Rojas, Claudia Duran-Aniotz, Adrian G. Palacios, Max Chacon, Leonel E. Medina
Summary: The study compared the complexity of healthy and diseased retinas at different ages using entropy tools, finding that young retinas have more complex responses to different visual stimuli, with wild-type retinas exhibiting significantly higher complexity in response to natural-like stimuli.
SCIENTIFIC REPORTS
(2022)
Article
Ophthalmology
Shannon R. Barwick, Haiyan Xiao, David Wolff, Jing Wang, Elizabeth Perry, Brendan Marshall, Sylvia B. Smith
Summary: Retinitis pigmentosa (RP) is a devastating inherited retinal disease that leads to visual impairment and blindness. Currently, there is no cure for RP, highlighting the importance of research into prolonging vision. This study investigated the effects of Sigma 1 receptor (Sig1R) activation in a mouse model of RP and found that it can protect visual function and structure. These results provide a framework for further studies on Sig1R as a potential therapeutic target for retinal degenerative diseases.
EXPERIMENTAL EYE RESEARCH
(2023)
Article
Cell Biology
Michael A. Robichaux, Vy Nguyen, Fung Chan, Lavanya Kailasam, Feng He, John H. Wilson, Theodore G. Wensel
Summary: The P23H-Rho-RFP mouse model provides a useful tool for studying the pathology and treatment of P23H-Rho and adRP. Mutant rod cells undergo an adaptive process that prolongs survival despite unfolded protein accumulation in the endoplasmic reticulum.
DISEASE MODELS & MECHANISMS
(2022)
Article
Multidisciplinary Sciences
Eva Halasz, Marco A. Zarbin, Amy L. Davidow, Laura J. Frishman, Peter Gombkoto, Ellen Townes-Anderson
Summary: Retinal detachment causes damage to the rod-bipolar synapse, which can disrupt vision. Using a pig model, researchers found that ROCK inhibition significantly reduced synaptic damage in detached retina, leading to improved functional outcomes after reattachment.
SCIENTIFIC REPORTS
(2021)
Article
Multidisciplinary Sciences
Christine Belloir, Marine Brule, Lucie Tornier, Fabrice Neiers, Loic Briand
Summary: Sweet taste perception is mediated by a heterodimeric receptor formed by TAS1R2 and TAS1R3 subunits. Researchers overexpressed and purified hTAS1R2 to study its structure and ligand binding properties, showing its capability to bind high potency sweeteners. This study provides a new experimental strategy for identifying new sweeteners or taste modulators acting on hTAS1R2, and is essential for future structural and biophysical studies.
SCIENTIFIC REPORTS
(2021)
Article
Biochemistry & Molecular Biology
Viviana Guadagni, Martina Biagioni, Elena Novelli, Paolo Aretini, Chiara Maria Mazzanti, Enrica Strettoi
Article
Cell Biology
Lara Testai, Eugenia Piragine, Ilaria Piano, Lorenzo Flori, Eleonora Da Pozzo, Vincenzo Miragliotta, Andrea Pirone, Valentina Citi, Lorenzo Di Cesare Mannelli, Simone Brogi, Sara Carpi, Alma Martelli, Paola Nieri, Claudia Martini, Carla Ghelardini, Claudia Gargini, Vincenzo Calderone
OXIDATIVE MEDICINE AND CELLULAR LONGEVITY
(2020)
Article
Neurosciences
Ilaria Piano, Vanessa D'Antongiovanni, Elena Novelli, Martina Biagioni, Michele Dei Cas, Rita Clara Paroni, Riccardo Ghidoni, Enrica Strettoi, Claudia Gargini
FRONTIERS IN NEUROSCIENCE
(2020)
Article
Nutrition & Dietetics
Ilaria Piano, Mattia Di Paolo, Francesca Corsi, Eugenia Piragine, Silvia Bisti, Claudia Gargini, Stefano Di Marco
Summary: Research has shown that retinal diseases can be caused by a variety of factors, with treatments still needing improvement, especially in the case of gene therapy. Current studies are mostly focused on animal models, with challenges in progressing to human clinical trials. By studying the treatment outcomes in different animal models, the effectiveness of each therapy can be better evaluated.
Article
Biochemistry & Molecular Biology
Debora Napoli, Martina Biagioni, Federico Billeri, Beatrice Di Marco, Noemi Orsini, Elena Novelli, Enrica Strettoi
Summary: The study reveals structural abnormalities in the RPE of RP patients, leading to dysfunction of the inner barrier. Findings also suggest a correlation between the extent of photoreceptor loss and interruption zones of ZO-1. Treatment with dexamethasone in RP mice not only rescues cones but also restores RPE structure, highlighting a potential feedback loop for simultaneous protection of cones and the RPE.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Carmelo Puglia, Debora Santonocito, Giuseppe Romeo, Sebastiano Intagliata, Giovanni Luca Romano, Enrica Strettoi, Elena Novelli, Carmine Ostacolo, Pietro Campiglia, Eduardo Maria Sommella, Rosario Pignatello, Claudio Bucolo
Summary: Lipid-based nanocarriers have been developed to improve the bioavailability of ophthalmic drugs, with a novel formulation LN-ODAF showing promising potential in diffusing through different ocular compartments and reaching the back of the eye.
Article
Multidisciplinary Sciences
Irem Kilicarslan, Lucia Zanetti, Elena Novelli, Christoph Schwarzer, Enrica Strettoi, Alexandra Koschak
Summary: Cav1.3 channels play a role in the degeneration of photoreceptors in rd10 retinas, with their absence delaying the central-to-peripheral degeneration process and potentially achieving temporary rescue of photoreceptors indirectly through other retinal cell layers.
SCIENTIFIC REPORTS
(2021)
Review
Biochemistry & Molecular Biology
Enrica Strettoi, Beatrice Di Marco, Noemi Orsini, Debora Napoli
Summary: This article reviews the plasticity of the retina, finding that it has limited plasticity outside the developmental stage. In contrast, the retina is highly adept at maintaining a stable structure and functional organization. The article also discusses the variable rate of structural plasticity in retinal neurons in different circumstances.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Neurosciences
Ilaria Piano, Francesca Corsi, Beatrice Polini, Claudia Gargini
Summary: Treatment with naringenin and quercetin can alleviate retinal degeneration by preserving photoreceptor functionality and increasing antioxidant and anti-apoptotic pathways.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Biochemistry & Molecular Biology
Lorenzo Chiaverini, Damiano Cirri, Iogann Tolbatov, Francesca Corsi, Ilaria Piano, Alessandro Marrone, Alessandro Pratesi, Tiziano Marzo, Diego La Mendola
Summary: Ammonium trichloro (dioxoethylene-O,O ') tellurate (AS101) is a potential therapeutic prodrug with immunomodulatory properties. This study investigates the activation process of AS101 and its analogues in the presence of water. The results demonstrate that water triggers the conversion of AS101 to the pharmacologically active species, and the effect is influenced by the halogens present. The study provides valuable insights into the chemical and mechanistic implications of AS101 activation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Francesca Corsi, Emma Baglini, Elisabetta Barresi, Silvia Salerno, Chiara Cerri, Claudia Martini, Federico Da Settimo Passetti, Sabrina Taliani, Claudia Gargini, Ilaria Piano
Summary: TSPO plays an important role in neuropathologies and brain injury. The study found that by inhibiting inflammatory and apoptotic processes, as well as reducing LPS-induced cellular cytotoxicity, targeting TSPO has potential for therapeutic treatment of inflammatory-based retinal neurodegeneration.
ACS CHEMICAL NEUROSCIENCE
(2022)
Article
Biochemistry & Molecular Biology
Mattia Di Paolo, Francesca Corsi, Maria Maggi, Luca Nardi, Silvia Bisti, Ilaria Piano, Claudia Gargini
Summary: Saffron treatment for retinal diseases depends on its molecular composition. Hydroponic cultivation can reduce costs and make this treatment more accessible.
Meeting Abstract
Anatomy & Morphology
Enrica Strettoi, Martina Biagioni, Federico Billeri, Elena Novelli, Debora Napoli
JOURNAL OF ANATOMY
(2021)
Article
Biochemistry & Molecular Biology
Felicia D'Andrea, Stefania Sartini, Ilaria Piano, Matteo Franceschi, Luca Quattrini, Lorenzo Guazzelli, Lidia Ciccone, Elisabetta Orlandini, Claudia Gargini, Concettina La Motta, Susanna Nencetti
JOURNAL OF ENZYME INHIBITION AND MEDICINAL CHEMISTRY
(2020)