Article
Nutrition & Dietetics
Gabrielle R. Phillips, Sarah E. Hancock, Andrew M. Jenner, Catriona McLean, Kelly A. Newell, Todd W. Mitchell
Summary: Huntington's disease is a late-onset genetic neurodegenerative disease that leads to cognitive, motor, and psychiatric impairments. It is caused by a polyQ mutation in the HTT gene, resulting in a polyglutamine expansion in the Huntingtin protein. Changes in phospholipids in the HD brain depend on the lipid subclass, species, bond type, and location.
Review
Multidisciplinary Sciences
Baljit S. Khakh, Steven A. Goldman
Summary: Huntington's disease (HD) is a fatal neurodegenerative disease caused by a mutation in the huntingtin gene. Astrocytes in the striatum, a brain region affected in HD, play a role in the pathology of the disease. Dysfunctions in astrocytes contribute to cellular and metabolic abnormalities in HD, suggesting the potential for therapeutic targeting of these cells to restore normal function.
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES
(2023)
Article
Neurosciences
Johannes Burtscher, Giuseppe Pepe, Federico Marracino, Luca Capocci, Susy Giova, Gregoire P. Millet, Alba Di Pardo, Vittorio Maglione
Summary: Huntington's disease (HD) is a rare hereditary neurodegenerative disorder characterized by defects in mitochondrial homeostasis and functions, including age-related changes in respiratory capacities. The study found differential expression of ETS components in the cortex and striatum of symptomatic R6/2 mice, indicating transcription, translation and/or mitochondrial import defects in R6/2 mouse brains.
Article
Endocrinology & Metabolism
Laura M. McNair, Graeme F. Mason, Golam M. Chowdhury, Lihong Jiang, Xiaoxian Ma, Douglas L. Rothman, Helle S. Waagepetersen, Kevin L. Behar
Summary: Anaplerosis, mediated by pyruvate carboxylase (PC) in astroglia, varies across different brain regions. The rate of PC contributes to oxidative glucose consumption and is positively correlated with glutamate/glutamine cycling.
JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM
(2022)
Article
Multidisciplinary Sciences
Florian Krach, Judith Stemick, Tom Boerstler, Alexander Weiss, Ioannis Lingos, Stephanie Reischl, Holger Meixner, Sonja Ploetz, Michaela Farrell, Ute Hehr, Zacharias Kohl, Beate Winner, Juergen Winkler
Summary: This study investigates the molecular mechanism of Branaplam, a modulator of alternative splicing, in Huntington's disease. The drug effectively lowers mutant HTT protein levels and improves alternative splicing pathology in patient-derived cellular models. These findings demonstrate the potential of splicing modulators in treating CAG repeat disorders.
NATURE COMMUNICATIONS
(2022)
Article
Neurosciences
Jacqueline M. Ehrman, Paloma Merchan-Sala, Lisa A. Ehrman, Bin Chen, Hee-Woong Lim, Ronald R. Waclaw, Kenneth Campbell
Summary: The projection neurons of the striatum can be categorized into the striatopallidal (indirect) pathway or the striatonigral (direct) pathway. Striatonigral axons pioneer the internal capsule and cerebral peduncle and provide guidance for corticofugal and thalamocortical axons. Defects in the striatonigral pathway can disrupt internal capsule formation.
JOURNAL OF NEUROSCIENCE
(2022)
Article
Biotechnology & Applied Microbiology
Melanie Alpaugh, Maria Masnata, Aurelie de Rus Jacquet, Eva Lepinay, Helena L. Denis, Martine Saint-Pierre, Peter Davies, Emmanuel Planel, Francesca Cicchetti
Summary: This study suggests that attenuating tau pathology could mitigate behavioral and molecular hallmarks associated with Huntington's disease.
Article
Neurosciences
Julien Chambon, Pragya Komal, Gil M. Lewitus, Gina M. Kemp, Simone Valade, Houaria Adaidi, Noura Al Bistami, David Stellwagen
Summary: Huntington's disease (HD) is a neurodegenerative disease caused by a polyglutamine expansion in the huntingtin gene. Neurodegeneration in HD first occurs in the striatum, accompanied by an elevation in inflammatory cytokines. In a mouse model of HD, early changes in synaptic input onto striatal medium spiny neurons were observed, including an increase in excitatory synaptic strength and a decrease in inhibitory synaptic strength, which are both dependent on the pro-inflammatory cytokine tumor necrosis factor alpha (TNF) signaling. These changes may contribute to the development of excitotoxicity during the progress of HD.
JOURNAL OF NEUROSCIENCE
(2023)
Article
Multidisciplinary Sciences
Foteini Paraskevopoulou, Poorya Parvizi, Gokce Senger, Nurcan Tuncbag, Christian Rosenmund, Ferah Yildirim
Summary: Transcriptional dysregulation in Huntington's disease (HD) leads to functional deficits in striatal neurons. Mutant Huntingtin (Htt) decreases synaptic output of striatal neurons autonomously, with a number of dysregulated genes linked to physiological deficiencies in mutant Htt neurons. Inhibiting histone deacetylase 1/3 activities rectifies functional and morphological deficits, as well as alleviates aberrant transcriptional profiles in mutant Htt neurons.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Review
Neurosciences
Melissa Serranilla, Melanie A. Woodin
Summary: The regulation of intracellular chloride levels is crucial for maintaining fast synaptic inhibition, which is primarily mediated by the cation-chloride cotransporters NKCC1 and KCC2. In healthy neurons, the expression of KCC2 is higher than NKCC1, resulting in lower levels of intracellular chloride. However, in immature neurons or neurological disorders, impaired KCC2 function and/or enhanced NKCC1 expression lead to chloride accumulation and GABA-mediated excitation. This review focuses on the role of chloride dysregulation in the healthy and Huntington's disease brain, with a particular emphasis on the basal ganglia circuitry and the potential therapeutic targets.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2022)
Article
Endocrinology & Metabolism
Beatriz Morte, Pilar Gil-Ibanez, Heike Heuer, Juan Bernal
Summary: The study suggests that the neurological impairment in AHDS cannot be fully explained by TH deprivation alone.
Article
Neuroimaging
Laurie Lemoine, Marine Lunven, Blanche Bapst, Laurent Cleret de Langavant, Vincent de Gardelle, Anne-Catherine Bachoud-Levi
Summary: HD patients show more impairment in the temporal domain compared to the spatial domain and exhibit greater temporal disorientation in daily life. In contrast, Pre-HD participants do not show specific temporal deficits. MRI analyses suggest that performance in temporal tasks is associated with larger striatal grey matter volume in gene carriers.
NEUROIMAGE-CLINICAL
(2021)
Article
Anatomy & Morphology
Vyshnavi Rallapalle, Annesha C. King, Michelle Gray
Summary: Huntington's disease is an adult-onset dominantly inherited neurodegenerative disease that affects the GABAergic medium spiny neurons in the striatum. Pathological changes in HD patient striatal tissue show a significant reduction in the number of parvalbumin-expressing interneurons.
FRONTIERS IN NEUROANATOMY
(2021)
Article
Neurosciences
angela Beatris Zemniacak, Rafael Teixeira Ribeiro, Camila Vieira Pinheiro, Samela de Azevedo Cunha, Tailine Quevedo Tavares, Ediandra Tissot Castro, Guilhian Leipnitz, Moacir Wajner, Alexandre Umpierrez Amaral
Summary: Maple syrup urine disease is caused by a deficiency in branched-chain alpha-keto acid dehydrogenase complex activity. This study found that intracerebroventricular injection of alpha-ketoisocaproic acid (KIC) disrupted redox homeostasis in the brain structures, leading to neurological damage. Antioxidants were able to attenuate these effects.
MOLECULAR NEUROBIOLOGY
(2023)
Article
Neurosciences
Hiroshi Makino
Summary: This study reveals that the brain is capable of composing a novel behavior by additively combining preacquired action-value representations with stochastic policies using a simple arithmetic operation. Empirical testing on mice demonstrates that subtask pretraining enhances learning of composite tasks.
NATURE NEUROSCIENCE
(2023)
Editorial Material
Clinical Neurology
Benjamin R. Miller, Daniel Strbian, Sophia Sundararajan
Article
Clinical Neurology
David J. Bonda, Sunil Manjila, Prachi Mehndiratta, Fahd Khan, Benjamin R. Miller, Kaine Onwuzulike, Gianfranco Puoti, Mark L. Cohen, Lawrence B. Schonberger, Ignazio Cali
NEUROSURGICAL FOCUS
(2016)
Article
Medicine, Research & Experimental
Rwoof Reshi, Chris Streib, Mustapha Ezzeddine, Michelle Biros, Benjamin Miller, Kamakshi Lakshminarayan, David Anderson, Agnieszka Ardelt
MEDICAL HYPOTHESES
(2017)
Review
Clinical Neurology
Vilakshan Alambyan, Jonathan Pace, Benjamin Miller, Mark L. Cohen, Sankalp Gokhale, Gagandeep Singh, Ming-Chieh Shun, Anthony Hammond, Ciro Ramos-Estebanez
Article
Orthopedics
Benjamin J. Miller, Raffi S. Avedian, Rajiv Rajani, Lee Leddy, Jeremy R. White, Judd Cummings, Tessa Balach, Kevin MacDonald
CLINICAL ORTHOPAEDICS AND RELATED RESEARCH
(2015)
Article
Orthopedics
Benjamin J. Miller, Rajiv Rajani, Lee Leddy, Emily E. Carmody Soni, Jeremy R. White
CLINICAL ORTHOPAEDICS AND RELATED RESEARCH
(2015)
Article
Biochemistry & Molecular Biology
Rebecca L. Scalzo, Garrett L. Peltonen, Scott E. Binns, Mahalakshmi Shankaran, Gregory R. Giordano, Dylan A. Hartley, Anna L. Klochak, Mark C. Lonac, Hunter L. R. Paris, Steve E. Szallar, Lacey M. Wood, Frederick F. Peelor, William E. Holmes, Marc K. Hellerstein, Christopher Bell, Karyn L. Hamilton, Benjamin F. Miller
Article
Chemistry, Multidisciplinary
Iwona E. Weidlich, Yuri Pevzner, Benjamin T. Miller, Igor V. Filippov, H. Lee Woodcock, Bernard R. Brooks
JOURNAL OF COMPUTATIONAL CHEMISTRY
(2015)
Article
Materials Science, Biomaterials
Amrita R. Yadav, Rashmi Sriram, Jared A. Carter, Benjamin L. Miller
MATERIALS SCIENCE & ENGINEERING C-MATERIALS FOR BIOLOGICAL APPLICATIONS
(2014)
Article
Health Care Sciences & Services
Stephen Petterson, Benjamin F. Miller, Jessica C. Payne-Murphy, Robert L. Phillips
FAMILIES SYSTEMS & HEALTH
(2014)
Article
Clinical Neurology
Ayham M. Alkhachroum, Benjamin Miller, Tarek Chami, Curtis Tatsuoka, Cathy Sila
JOURNAL OF CLINICAL NEUROSCIENCE
(2019)
Letter
Biophysics
Nuttavut Sumransub, Fiona He, Daniel Weisdorf, Charles J. Billington, Anjali Aggarwal, Benjamin Miller, Veronika Bachanova
BONE MARROW TRANSPLANTATION
(2022)
Article
Biology
Ivana Labounkova, Rene Labounek, Radim Kolar, Ralf P. Tornow, Charles F. Babbs, Collin M. McClelland, Benjamin R. Miller, Igor Nestrasil
Summary: Video-recordings of retinal pulsatile patterns in the human eye are influenced by heart rate and age, which has significant implications for the modeling of retinal function and clinical design across various medical specialties.
COMMUNICATIONS BIOLOGY
(2022)
Article
Medicine, General & Internal
Malik Ghannam, Dana Ghazateh, Azizuttah Beran, Benjamin Miller, Brent Berry
AMERICAN JOURNAL OF CASE REPORTS
(2019)
Letter
Clinical Neurology
Ciro Ramos-Estebanez, Jonathan Pace, Sankalp Gokhale, Benjamin Miller, Sunil Manjila
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.