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Huntington disease

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https://www.readbyqxmd.com/read/27929218/emerging-vector-borne-diseases
#1
Mark K Huntington, Jay Allison, Dilip Nair
Several mosquito-borne viral infections have recently emerged in North America; West Nile virus is the most common in the United States. Although West Nile virus generally causes a self-limited, flulike febrile illness, a serious neuroinvasive form may occur. Dengue is the most common vector-borne viral disease worldwide, and it has been a significant public health threat in the United States since 2009. Known as breakbone fever for its severe myalgias and arthralgias, dengue may cause a hemorrhagic syndrome...
October 1, 2016: American Family Physician
https://www.readbyqxmd.com/read/27928694/effect-of-rutin-against-a-mitochondrial-toxin-3-nitropropionicacid-induced-biochemical-behavioral-and-histological-alterations-a-pilot-study-on-huntington-s-disease-model-in-rats
#2
Sarumani Natarajan Suganya, Thangarajan Sumathi
Dietary compounds like flavonoids may offer protection against neurodegeneration. Huntington's disease (HD) is a neurodegenerative disorder characterized by symptoms like chorea and dementia. 3-Nitropropionic acid (3-NP), a Succinate dehydrogenase (SDH) inhibitor produces behavioral, biochemical and histological changes in the striatum, mimics HD in animals and humans. The present study was designed to examine the protective activity of Rutin (RT), a primary flavonoid from citrus fruits, green tea on 3-NP induced experimental model of HD in rats...
December 8, 2016: Metabolic Brain Disease
https://www.readbyqxmd.com/read/27927647/gvhd-prevents-nk-cell-dependent-leukemia-and-virus-specific-innate-immunity
#3
Mark D Bunting, Antiopi Varelias, Fernando Souza-Fonseca-Guimaraes, Iona S Schuster, Katie E Lineburg, Rachel D Kuns, Peter Fleming, Kelly R Locke, Nicholas D Huntington, Bruce R Blazar, Steven W Lane, Siok-Keen Tey, Kelli P A MacDonald, Mark J Smyth, Mariapia A Degli-Esposti, Geoffrey R Hill
Allogeneic bone marrow transplantation (allo-BMT) is a curative therapy for hematological malignancies, but is associated with significant complications, principally graft-versus-host disease (GVHD) and opportunistic infections. Natural killer (NK) cells mediate important innate immunity that provides a temporal bridge until the reconstruction of adaptive immunity. Here we show that the development of GVHD after allo-BMT prevented NK-cell reconstitution, particularly within the maturing M1 and M2 NK-cell subsets in association with exaggerated activation, apoptosis and autophagy...
December 7, 2016: Blood
https://www.readbyqxmd.com/read/27927242/sudden-death-due-to-paralysis-and-synaptic-and-behavioral-deficits-when-hip14-zdhhc17-is-deleted-in-adult-mice
#4
Shaun S Sanders, Matthew P Parsons, Katherine K N Mui, Amber L Southwell, Sonia Franciosi, Daphne Cheung, Sabine Waltl, Lynn A Raymond, Michael R Hayden
BACKGROUND: Palmitoylation, the addition of palmitate to proteins by palmitoyl acyltransferases (PATs), is an important regulator of synaptic protein localization and function. Many palmitoylated proteins and PATs have been implicated in neuropsychiatric diseases, including Huntington disease, schizophrenia, amyotrophic lateral sclerosis, Alzheimer disease, and X-linked intellectual disability. HIP14/DHHC17 is the most conserved PAT that palmitoylates many synaptic proteins. Hip14 hypomorphic mice have behavioral and synaptic deficits...
December 7, 2016: BMC Biology
https://www.readbyqxmd.com/read/27927041/investigational-agents-for-the-management-of-huntington-s-disease
#5
Thomas Müller
An inherited, chronic progressive, neurodegenerative disorder is Huntington's disease, characterized by motor, cognitive, and psychiatric symptoms. Predictive genetic testing allows earlier diagnosis and identification of gene carriers for Huntington's disease. These individuals are ideal candidates for testing of therapeutic interventions for disease modification. Areas covered: According to queries in Pubmed, Embase and clinical register databases, research and clinical studies emerge on symptomatic and neuroprotective therapies in Huntington's disease...
December 7, 2016: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/27924526/the-p-r151c-polymorphism-in-mc1r-gene-modifies-the-age-of-onset-in-spanish-huntington-s-disease-patients
#6
Gemma Tell-Marti, Joan Anton Puig-Butille, Pol Gimenez-Xavier, Ariadna Segu-Roig, Miriam Potrony, Celia Badenas, Victoria Alvarez, José M Millán, María José Trujillo-Tiebas, María A Ramos-Arroyo, Montserrat Milà, Susana Puig
The expansion of CAG repeats (≥36 CAG) in the HTT gene is the only known genetic cause of Huntington's disease (HD) and the main determinant of the course of the disease. The length of the expanded CAG repeats correlates inversely with the age of onset (AOO) but does not completely determine it. We investigated the role of the melanocortin 1 receptor (MC1R) gene as a modifier factor of AOO in 600 HD patients from Spain. We sequenced the entire region of the MC1R gene and analyzed all the nonsynonymous MC1R genetic variants with a minor allele frequency of at least 0...
December 6, 2016: Molecular Neurobiology
https://www.readbyqxmd.com/read/27924204/combined-use-of-a-double-lumen-tube-and-fogarty-catheter-to-prevent-the-endobronchial-spread-of-infection-a-case-report
#7
Jaewon Kim, Hyelim Lee, Han Park, Chang-Young Jeong
Huntington's disease is a neurodegenerative disorder with an autosomal dominant inheritance pattern. Patients with Huntington's disease show an increased risk of aspiration pneumonia when the pharyngeal muscle is invaded. We report a case of advanced-stage Huntington's disease in which the patient received right middle lobectomy for a lung abscess caused by repeated aspiration. The best lung isolation technique has not yet been established in these patients. We successfully performed selective lobar isolation of the right lower and middle lobes using a double lumen tube and a Fogarty embolectomy catheter...
December 2016: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/27924190/network-analysis-of-human-post-mortem-microarrays-reveals-novel-genes-micrornas-and-mechanistic-scenarios-of-potential-importance-in-fighting-huntington-s-disease
#8
Sreedevi Chandrasekaran, Danail Bonchev
Huntington's disease is a progressive neurodegenerative disorder characterized by motor disturbances, cognitive decline, and neuropsychiatric symptoms. In this study, we utilized network-based analysis in an attempt to explore and understand the underlying molecular mechanism and to identify critical molecular players of this disease condition. Using human post-mortem microarrays from three brain regions (cerebellum, frontal cortex and caudate nucleus) we selected in a four-step procedure a seed set of highly modulated genes...
2016: Computational and Structural Biotechnology Journal
https://www.readbyqxmd.com/read/27922200/a-fluorescence-lifetime-based-binding-assay-for-class-iia-histone-deacetylases
#9
Christian Meyners, Monique Mertens, Pablo Wessig, Franz-Josef Meyer-Almes
Class IIa HDACs show extremely low enzymatic activity and no commonly accepted endogenous substrate is known today. Increasing evidence suggests that these enzymes exert their effect rather through molecular recognition of acetylated proteins and recruiting other proteins like HDAC3 to the desired target location. Accordingly, class IIa HDACs like bromodomains have been suggested to act as "Readers" of acetyl marks, whereas enzymatically active HDACs from class I or IIb are called "Erasers" to highlight their capability to remove acetyl groups from acetylated histones or other proteins...
December 6, 2016: Chemistry: a European Journal
https://www.readbyqxmd.com/read/27920668/getting-mirna-therapeutics-into-the-target-cells-for-neurodegenerative-diseases-a-mini-review
#10
REVIEW
Ming Ming Wen
miRNAs play important roles in modulating gene expression in varying cellular processes and disease pathogenesis, including neurodegenerative diseases. Several miRNAs are expressed in the brain, control brain development and are identified as important biomarkers in the pathogenesis of motor-and neuro-cognitive diseases such as Alzheimer's (AD), Huntington's and Parkinson's diseases (PD) and amyotrophic lateral sclerosis. These remarkable miRNAs could be used as diagnostic markers and therapeutic targeting potential for many stressful and untreatable progressive neurodegenerative diseases...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27917685/phosphodiesterase-10-inhibitors-in-clinical-development-for-cns-disorders
#11
Hugo Geerts, Athan Spiros, Patrick Roberts
Phosphodiesterase 10 inhibitors (PDE10-I), are conceptually attractive drugs with a potential great therapeutic window as their enriched striatal localization may likely stimulate D1R and reduce D2R downstream effects. However, so far selective PDE10-I with efficacy in animal models have not shown benefit in clinical trials and unexpectedly revealed a substantial dyskinesia motor side-effect. Areas covered: This paper reviews the underlying biological rationale of PDE10 as a target in schizophrenia, Parkinson's and Huntington's disease based on peer-reviewed published articles, the status of the different PDE10-I in clinical development for various CNS indications and explores possible reasons for the clinical trial failures and translational disconnect...
December 3, 2016: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/27917499/longitudinal-beta-binomial-modeling-using-gee-for-overdispersed-binomial-data
#12
Hongqian Wu, Ying Zhang, Jeffrey D Long
Longitudinal binomial data are frequently generated from multiple questionnaires and assessments in various scientific settings for which the binomial data are often overdispersed. The standard generalized linear mixed effects model may result in severe underestimation of standard errors of estimated regression parameters in such cases and hence potentially bias the statistical inference. In this paper, we propose a longitudinal beta-binomial model for overdispersed binomial data and estimate the regression parameters under a probit model using the generalized estimating equation method...
December 5, 2016: Statistics in Medicine
https://www.readbyqxmd.com/read/27917451/reversal-learning-reveals-cognitive-deficits-and-altered-prediction-error-encoding-in-the-ventral-striatum-in-huntington-s-disease
#13
Katharina Nickchen, Rebecca Boehme, Maria Del Mar Amador, Thomas D Hälbig, Katharina Dehnicke, Patricia Panneck, Joachim Behr, Konstantin Prass, Andreas Heinz, Lorenz Deserno, Florian Schlagenhauf, Josef Priller
Huntington's disease (HD) is an autosomal dominant neurodegenerative condition characterized by a triad of movement disorder, neuropsychiatric symptoms and cognitive deficits. The striatum is particularly vulnerable to the effects of mutant huntingtin, and cell loss can already be found in presymptomatic stages. Since the striatum is well known for its role in reinforcement learning, we hypothesized to find altered behavioral and neural responses in HD patients in a probabilistic reinforcement learning task performed during functional magnetic resonance imaging...
December 5, 2016: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/27917304/quality-of-life-of-cypriot-patients-suffering-with-huntington-s-disease
#14
Eleni Varda, Christiana A Demetriou, Alexandros Heraclides, Yiolanda P Christou, Eleni Zamba-Papanicolaou
INTRODUCTION: Huntington's disease (HD) has profound motor, behavioural and cognitive symptoms. Despite the enormous burden of this disease on the quality of life (QoL) of patients and their families, there is very limited evidence on this topic. Considering the severity of HD patients, and the high prevalence in Cyprus more studies are needed to assess QoL among Cypriot patients, in order to improve our knowledge about their living conditions and to assist the management of this condition...
October 25, 2016: PLoS Currents
https://www.readbyqxmd.com/read/27916455/phosphodiesterase-10a-inhibition-improves-cortico-basal-ganglia-function-in-huntington-s-disease-models
#15
Vahri Beaumont, Sheng Zhong, Hai Lin, WenJin Xu, Amyaouch Bradaia, Esther Steidl, Melanie Gleyzes, Kristian Wadel, Bruno Buisson, Fernando E Padovan-Neto, Shreaya Chakroborty, Karen M Ward, John F Harms, Jose Beltran, Mei Kwan, Afshin Ghavami, Jenny Häggkvist, Miklós Tóth, Christer Halldin, Andrea Varrone, Christoph Schaab, J Nikolaj Dybowski, Sarah Elschenbroich, Kimmo Lehtimäki, Taneli Heikkinen, Larry Park, James Rosinski, Ladislav Mrzljak, Daniel Lavery, Anthony R West, Christopher J Schmidt, Margaret M Zaleska, Ignacio Munoz-Sanjuan
Huntington's disease (HD) symptoms are driven to a large extent by dysfunction of the basal ganglia circuitry. HD patients exhibit reduced striatal phoshodiesterase 10 (PDE10) levels. Using HD mouse models that exhibit reduced PDE10, we demonstrate the benefit of pharmacologic PDE10 inhibition to acutely correct basal ganglia circuitry deficits. PDE10 inhibition restored corticostriatal input and boosted cortically driven indirect pathway activity. Cyclic nucleotide signaling is impaired in HD models, and PDE10 loss may represent a homeostatic adaptation to maintain signaling...
November 25, 2016: Neuron
https://www.readbyqxmd.com/read/27915242/distinct-mechanisms-of-mutant-huntingtin-toxicity-in-different-yeast-strains
#16
Genrikh V Serpionov, Alexander I Alexandrov, Michael D Ter-Avanesyan
Expansion of polyglutamine stretches in several proteins cause neurodegenerative amyloidoses, including Huntington disease. In yeast, mutant huntingtin (mHtt) with a stretch of 103 glutamine residues (HttQ103) forms toxic aggregates. A range of yeast strains have been used to elucidate the mechanisms of mHtt toxicity, and have revealed perturbations of various unrelated processes. HttQ103 aggregates can induce aggregation of cellular proteins, many of which contain glutamine/asparagine-rich regions, including Sup35 and Def1...
December 2, 2016: FEMS Yeast Research
https://www.readbyqxmd.com/read/27913697/is-mitochondrial-oxidative-metabolism-the-right-therapy-target-in-early-huntington-disease
#17
Raffaele Lodi
No abstract text is available yet for this article.
December 2, 2016: Neurology
https://www.readbyqxmd.com/read/27913695/a-randomized-double-blind-placebo-controlled-trial-of-coenzyme-q10-in-huntington-disease
#18
Andrew McGarry, Michael McDermott, Karl Kieburtz, Elisabeth A de Blieck, Flint Beal, Karen Marder, Christopher Ross, Ira Shoulson, Peter Gilbert, William M Mallonee, Mark Guttman, Joanne Wojcieszek, Rajeev Kumar, Mark S LeDoux, Mary Jenkins, H Diana Rosas, Martha Nance, Kevin Biglan, Peter Como, Richard M Dubinsky, Kathleen M Shannon, Padraig O'Suilleabhain, Kelvin Chou, Francis Walker, Wayne Martin, Vicki L Wheelock, Elizabeth McCusker, Joseph Jankovic, Carlos Singer, Juan Sanchez-Ramos, Burton Scott, Oksana Suchowersky, Stewart A Factor, Donald S Higgins, Eric Molho, Fredy Revilla, John N Caviness, Joseph H Friedman, Joel S Perlmutter, Andrew Feigin, Karen Anderson, Ramon Rodriguez, Nikolaus R McFarland, Russell L Margolis, Eric S Farbman, Lynn A Raymond, Valerie Suski, Sandra Kostyk, Amy Colcher, Lauren Seeberger, Eric Epping, Sherali Esmail, Nancy Diaz, Wai Lun Alan Fung, Alan Diamond, Samuel Frank, Philip Hanna, Neal Hermanowicz, Leon S Dure, Merit Cudkowicz
OBJECTIVE: To test the hypothesis that chronic treatment of early-stage Huntington disease (HD) with high-dose coenzyme Q10 (CoQ) will slow the progressive functional decline of HD. METHODS: We performed a multicenter randomized, double-blind, placebo-controlled trial. Patients with early-stage HD (n = 609) were enrolled at 48 sites in the United States, Canada, and Australia from 2008 to 2012. Patients were randomized to receive either CoQ 2,400 mg/d or matching placebo, then followed for 60 months...
December 2, 2016: Neurology
https://www.readbyqxmd.com/read/27913616/genetic-contributors-to-intergenerational-cag-repeat-instability-in-huntington-s-disease-knock-in-mice
#19
João Luís Neto, Jong-Min Lee, Ali Afridi, Tammy Gillis, Jolene R Guide, Stephani Dempsey, Brenda Lager, Isabel Alonso, Vanessa C Wheeler, Ricardo Mouro Pinto
Huntington's disease is a neurodegenerative disorder caused by the expansion of a CAG trinucleotide repeat in exon 1 of the HTT gene. Longer repeat sizes are associated with increased disease penetrance and earlier ages of onset. Intergenerationally unstable transmissions are common in Huntington's disease families, partly underlying the genetic anticipation seen in this disorder. Huntington's disease CAG knock-in mouse models also exhibit a propensity for intergenerational repeat size changes. In this work, we examine intergenerational instability of the CAG repeat in over 20,000 transmissions in the largest Huntington's disease knock-in mouse model breeding datasets reported to date...
December 2, 2016: Genetics
https://www.readbyqxmd.com/read/27913290/early-and-brain-region-specific-decrease-of-de-novo-cholesterol-biosynthesis-in-huntington-s-disease-a-cross-validation-study-in-q175-knock-in-mice
#20
Mahalakshmi Shankaran, Eleonora Di Paolo, Valerio Leoni, Claudio Caccia, Costanza Ferrari Bardile, Hussein Mohammed, Stefano Di Donato, Seung Kwak, Deanna Marchionini, Scott Turner, Elena Cattaneo, Marta Valenza
Cholesterol precursors and cholesterol levels are reduced in brain regions of Huntington's disease (HD) mice. Here we quantified the rate of in vivo de novo cholesterol biosynthesis in the HD brain. Samples from different brain regions and blood of the heterozygous knock-in mouse model carrying 175 CAG repeats (Q175) at different phenotypic stages were processed independently by two research units to quantify cholesterol synthesis rate by (2)H2O labeling and measure the concentrations of lathosterol, cholesterol and its brain-specific cholesterol catabolite 24-hydroxy-cholesterol (24OHC) by isotope dilution mass spectrometry...
November 30, 2016: Neurobiology of Disease
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