Article
Cell Biology
Lucie Crouzier, Camille Diez, Elodie M. Richard, Nicolas Cubedo, Clement Barbereau, Mireille Rossel, Thomas Delaunay, Tangui Maurice, Benjamin Delprat
Summary: Retinitis pigmentosa (RP) is a common inherited retinal degeneration disease with no current treatment available. The zebrafish pde6a(Q70X) mutant serves as an ideal model for studying the progression of RP and potential treatments.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Multidisciplinary Sciences
Ayoub Lassoued, Furu Zhang, Kazuhiro Kurokawa, Yan Liu, Marcel T. Bernucci, James A. Crowell, Donald T. Miller
Summary: Retinitis pigmentosa (RP) is a group of inherited retinal degenerative diseases, with cone photoreceptor death being the most debilitating phase. High-resolution retinal imaging methods and cone optoretinogram show promise in visualizing individual cone cells and their dysfunction in RP progression.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Multidisciplinary Sciences
Yunlu Xue, Xiaomei Sun, Sean K. Wang, Gayle B. Collin, Vladimir J. Kefalov, Constance L. Cepko
Summary: Retinitis pigmentosa (RP) is an ocular disease characterized by the loss of night vision, followed by the loss of daylight vision. The timing of cone electroretinogram (ERG) decline in RP mouse models was investigated using physiological assays, and a correlation with the loss of rods was found. Abnormally high chromophore supply to cones upon the loss of rods is toxic to cones, suggesting a potential therapy in some forms of RP to slow down the turnover or reduce the level of visual chromophore in the retina.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2023)
Article
Cell & Tissue Engineering
Eric D. Jong, Sabiha Hacibekiroglu, Lily Guo, Evan Sawula, Biao Li, Chengjin Li, Margaret T. Ho, Molly S. Shoichet, Valerie A. Wallace, Andras Nagy
Summary: A combined cell and gene therapy was developed for the treatment of retinitis pigmentosa (RP). Human embryonic stem cells (hESCs) were genetically engineered to differentiate into retinal pigment epithelium (hRPE) cells, which were used to protect and preserve photoreceptor cells in mouse models. This study reveals the potential of this therapy for RP treatment and the possibility of using hRPE cells for long-term delivery of therapeutic biologics.
STEM CELL RESEARCH & THERAPY
(2023)
Article
Biology
Duygu Karademir, Vyara Todorova, Lynn J. A. Ebner, Marijana Samardzija, Christian Grimm
Summary: This study utilizes droplet-based single-cell RNA sequencing to investigate the degeneration process in rod and cone photoreceptor cells in an RP mouse model. The results identify early changes in rods and potential early responses in cones, suggesting mechanisms involved in secondary cone degeneration. Furthermore, the study implicates EGR1 as a potential key regulator of early degenerative events in both types of photoreceptor cells.
Article
Ophthalmology
Paul. S. Micevych, Jessica Wong, Hao Zhou, Ruikan. K. Wang, Travis. C. Porco, Joseph Carroll, Austin Roorda, Jacque. L. Duncan
Summary: This study compared cone structure and function between patients with RPGR and USH2A-related retinal degeneration. It found that outer retinal structure and choriocapillaris perfusion were more abnormal in patients with RPGR-related retinal degeneration compared to those with USH2A-related retinal degeneration. However, there were no significant differences in below-normal regional sensitivity between rod-cone degeneration associated with variants in these 2 genes expressed at the photoreceptor-connecting cilium.
AMERICAN JOURNAL OF OPHTHALMOLOGY
(2023)
Article
Multidisciplinary Sciences
Satoru Kanda, Takumi Hara, Ryosuke Fujino, Keiko Azuma, Hirotsugu Soga, Ryo Asaoka, Ryo Obata, Tatsuya Inoue
Summary: The study found that measuring the low FAF area is very useful for patients with cone-rod dystrophy. AF measurement reflects visual field damage, but not visual acuity (VA).
SCIENTIFIC REPORTS
(2021)
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
Biology
Miranda L. Scalabrino, Mishek Thapa, Lindsey A. Chew, Esther Zhang, Jason Xu, Alapakkam P. Sampath, Jeannie Chen, Greg D. Field
Summary: The impact of progressive rod death on cone-mediated signaling remains unclear. Using a mouse model, researchers found that despite deterioration in cone morphology, the cone-mediated signaling among RGCs remained relatively stable throughout disease progression, until almost all rods had died and cones had completely lost outer segments.
Article
Ophthalmology
Danial Roshandel, Rachael C. Heath Jeffery, Jason Charng, Danuta M. Sampson, Samuel McLenachan, David A. Mackey, Fred K. Chen
Summary: The study investigated parafoveal cone loss in patients with RCD using flood-illumination adaptive optics imaging. Results showed significant cone loss over a 6-month follow-up period even in regions with preserved EZ, indicating the potential of cone metrics as a structural endpoint in early RCD.
TRANSLATIONAL VISION SCIENCE & TECHNOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Renske Schellens, Erik de Vrieze, Pam Graave, Sanne Broekman, Kerstin Nagel-Wolfrum, Theo Peters, Hannie Kremer, Rob W. J. Collin, Erwin van Wijk
Summary: Investigating the therapeutic potential of exon skipping for EYS-associated RP, this study employed CRISPR/Cas9 to generate zebrafish with the deletion of exons 37-41 of human EYS. However, the results from this zebrafish model do not provide indications for skipping EYS exons 37-41 as an effective future treatment strategy for EYS-associated RP.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Yuqin Liang, Feng Tan, Xihao Sun, Zekai Cui, Jianing Gu, Shengru Mao, Hon Fai Chan, Shibo Tang, Jiansu Chen
Summary: This study established an iPSCs model of a PRPF6-related adRP patient and generated a disease model of RPE cells, revealing the effects of PRPF6 mutation on the morphology, gene expression, and function of RPE cells. This research is of great significance for understanding PRPF6-related RPE cells and future cell therapy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Multidisciplinary Sciences
Benedetto Falsini, Giorgio Placidi, Elisa De Siena, Pietro Chiurazzi, Angelo Maria Minnella, Maria Cristina Savastano, Lucia Ziccardi, Vincenzo Parisi, Giancarlo Iarossi, Marcella Percio, Barbora Pitekova, Giuseppe Marceddu, Paolo Enrico Maltese, Matteo Bertelli
Summary: This study examined Italian patients with macular, cone and cone-rod dystrophies using next-generation sequencing, gene sequencing panels, and other techniques to diagnose the molecular cause of the diseases. The diagnostic yield was high, and the main cause of the diseases was found to be variants in the ABCA4 gene. The study highlights the importance of targeted NGS and genetic counseling in diagnosing these diseases.
SCIENTIFIC REPORTS
(2022)
Article
Neurosciences
Christopher M. Whitaker, Gina Nobles, Munenori Ishibashi, Stephen C. Massey
Summary: Rod and cone pathways are initially segregated in the retina, but there is evidence of some crossover connections between the two pathways. Physiological recordings suggest that rod bipolar cells can receive input from cones, and ON cone bipolar cells may also receive input from rods. Imaging studies show that some rod bipolar cells have cone contacts, while tertiary rod pathways operate via direct contacts between rods and OFF cone bipolar cells. However, no evidence was found for rod contacts with ON cone bipolar cells in the rabbit retina.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2021)
Article
Neurosciences
Pablo Beltran-Matas, Espen Hartveit, Margaret L. Veruki
Summary: The NMDA receptors expressed by AII and A17 amacrine cells in the mammalian retina are extrasynaptic and molecularly distinct, being activated by different sources of glutamate and endogenous co-agonists. The differential and independent activation of these receptors likely play specific roles in signal processing and plasticity of the rod pathway microcircuit.
EUROPEAN JOURNAL OF NEUROSCIENCE
(2021)
Article
Neurosciences
Xiaomei Lin, Tianyuyi Feng, Erheng Cui, Yunfei Li, Zhang Qin, Xiaohu Zhao
Summary: This study successfully established a rat model based on the genetic-environmental interaction, which exhibited phenotype characteristics similar to human AD in terms of cognitive function, brain microstructure, and immunohistochemistry. The genetic factor (APP mutation) and the environmental factor (acrolein exposure) accounted for 39.74% and 33.3% of the AD-like phenotypes in the model, respectively.
Article
Neurosciences
Gustavo Guimara Guerrero, Giovanna Bignoto Minhoto, Camilla dos Santos Tiburcio-Machado, Itza Amarisis Ribeiro Pinto, Claudio Antonio Federico, Marcia Carneiro Valera
Summary: The present study evaluated the influence of head and neck radiotherapy on the behavior and body weight gain in Wistar rats. The results demonstrated that different doses of radiation induced depressive behavior in the animals, and that the weight gain tended to be lower in the irradiated groups.
Article
Neurosciences
Ziwei Gao, Chao Lu, Yaping Zhu, Yuxin Liu, Yuesong Lin, Wenming Gao, Liyuan Tian, Lei Wu
Summary: This study reveals the underlying mechanisms of the rapid antidepressant effects of merazin hydrate (MH), which activates CaMKII to promote neuronal activities and proliferation in the hippocampus.
Article
Neurosciences
Kathleen E. Murray, Whitney A. Ratliff, Vedad Delic, Bruce A. Citron
Summary: Gulf War Illness (GWI) is a chronic disorder that affects approximately 30% of Veterans deployed to the Persian Gulf. This study found that exposure to toxicants during the Gulf War resulted in long-term changes in the morphology of dentate granule cells and that treatment with Nrf2 activator could improve neuronal health in the hippocampus.
Article
Neurosciences
Jing Li, Yan Zou, Xiangchuang Kong, Yangming Leng, Fan Yang, Guofeng Zhou, Bo Liu, Wenliang Fan
Summary: This study examines the functional connectivity changes in individuals with sudden sensorineural hearing loss (SSNHL) at the integrity, network, and edge levels. The findings reveal reduced intranetwork connectivity strength and increased internetwork connectivity in SSNHL patients. These alterations are associated with the duration of SSNHL and Tinnitus Handicap Inventory scores. The study provides crucial insights into the neural mechanisms of SSNHL and the brain's network-level responses to sensory loss.
Review
Neurosciences
Didier Majou, Anne-Lise Dermenghem
Summary: In the early stages of SAD, memory impairment is strongly correlated with cortical levels of soluble amyloid-beta peptide oligomers. A beta disrupts glutamatergic synaptic function and leads to cognitive deficits. This article describes the pathogenic mechanisms underlying cerebral amyloidosis, involving amyloid precursor protein synthesis, A beta residue clearance processes, and the role of specific molecules.
Article
Neurosciences
Jing Li, Yi Shan, Xiaojing Zhao, Guixiang Shan, Peng-Hu Wei, Lin Liu, Changming Wang, Hang Wu, Weiqun Song, Yi Tang, Guo-Guang Zhao, Jie Lu
Summary: This study investigates changes in brain anatomical structures and functional network connectivity after chronic complete thoracic spinal cord injury (cctSCI) and their impact on clinical outcomes. The findings reveal alterations in gray matter volume and functional connectivity in specific brain regions, indicating potential therapeutic targets and methods for tracking treatment outcomes.
Article
Neurosciences
Anllely Fernandez, Katherine Corvalan, Octavia Santis, Maxs Mendez-Ruette, Ariel Caviedes, Matias Pizarro, Maria -Teresa Gomez, Luis Federico Batiz, Peter Landgraf, Thilo Kahne, Alejandro Rojas-Fernandez, Ursula Wyneken
Summary: This study reveals the importance of SUMOylation in modulating the protein cargo of astrocyte-derived small extracellular vesicles (sEVs) and its potential impact on neurons.
Article
Neurosciences
Anika Luettig, Stefanie Perl, Maria Zetsche, Franziska Richter, Denise Franz, Marco Heerdegen, Ruediger Koehling, Angelika Richter
Summary: This study found that changes in c-Fos activity during short-term stimulation of the entopeduncular nucleus (EPN) are associated with improvement in dystonia, and also discovered that the cerebellum may be involved in the antidystonic effects.
Article
Neurosciences
Yanlin Tao, Wei Shen, Houyuan Zhou, Zikang Li, Ting Pi, Hui Wu, Hailian Shi, Fei Huang, Xiaojun Wu
Summary: Depression has a higher incidence in women compared to men, and this study investigated the impact of sex on depressive behaviors and underlying mechanisms using a corticosterone-induced depression model in mice. The results showed sex-specific anxiety and depression behaviors in the model group, as well as differences in protein expression and neurotransmitter levels between male and female mice. These findings enhance our understanding of sex-specific differences in depression and support tailored interventions.
Review
Neurosciences
Dnyandev G. Gadhave, Vrashabh V. Sugandhi, Chandrakant R. Kokare
Summary: This article discusses the characteristics and importance of the tight junctions of endothelial cells in the CNS, which act as a biological barrier known as the blood-brain barrier (BBB). It focuses on overcoming the challenges of delivering therapeutic agents to the brain in neurodegenerative disorders, particularly multiple sclerosis, through the use of biomaterials. The article also highlights the current limitations of animal models for studying multiple sclerosis and suggests a potential future research direction.
Article
Neurosciences
Li-Min Mao, Khyathi Thallapureddy, John Q. Wang
Summary: Propofol can enhance synapsin phosphorylation and modulate synaptic transmission in the mouse brain. The study reveals the potential role of synapsin as a substrate of propofol and its effects on neurotransmitter release machinery.
Article
Neurosciences
Syed Maaz Ahmed Rizvi, Abdul Baseer Buriro, Irfan Ahmed, Abdul Aziz Memon
Summary: This study explores the effects of prolonged mask usage on the human brain by analyzing EEG and physiological parameters. The results show that the mean EEG spectral power in alpha, beta, and gamma sub-bands of individuals wearing masks is smaller than those without masks. The performances on cognitive tasks and oxygen saturation level differ between the two groups, while blood pressure, body temperature, and heart rate are similar. The analysis also reveals that the occipital and frontal lobes exhibit the greatest variability in channel measurements.
Article
Neurosciences
Rui-Fang Ma, Lu-Lu Xue, Jin-Xiang Liu, Li Chen, Liu-Lin Xiong, Ting-Hua Wang, Fei Liu
Summary: This study observed changes in brain infarction and blood vessels in rats during neonatal hypoxic ischemic encephalopathy (NHIE) modeling using Transcranial Doppler Ultrasonography (TCD). Longer duration of hypoxia was associated with more severe nerve damage. TCD can dynamically monitor cerebral infarction after NHIE modeling, which may serve as a useful auxiliary method for evaluating animal experimental models.
Article
Neurosciences
Yuxiang Dai, Chen Yu, Lu Zhou, Longyang Cheng, Hongbin Ni, Weibang Liang
Summary: Overexpression of CXCR4 in glioma is correlated with patient survival, and its inhibition can reduce invasion and migration of glioma cells. Inhibiting Nur77 also decreases cancer progression associated with CXCR4.