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https://www.readbyqxmd.com/read/29676484/structural-and-metabolic-brain-correlates-of-apathy-in-huntington-s-disease
#1
Saul Martínez-Horta, Jesús Perez-Perez, Frederic Sampedro, Javier Pagonabarraga, Andrea Horta-Barba, Mar Carceller-Sindreu, Beatriz Gomez-Anson, Gloria Andrea Lozano-Martinez, Diego Alfonso Lopez-Mora, Valle Camacho, Alejandro Fernández-León, Ignasi Carrió, Jaime Kulisevsky
BACKGROUND: Apathy is the most prevalent and characteristic neuropsychiatric feature of Huntington's disease. Congruent with the main early pathological changes, apathy is primarily associated with subcortical damage in frontal-striatal circuits. However, little is known about its precise subserving mechanisms and the contribution of regions other than the basal ganglia. OBJECTIVES: We aimed to define the neural correlates of apathy in Huntington's disease based on gray matter volume and PET/CT of 18 F-fluorodeoxyglucose metabolism...
April 20, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29676231/combating-neurodegenerative-diseases-with-the-plant-alkaloid-berberine-molecular-mechanisms-and-therapeutic-potential
#2
Dahua Fan, Liping Liu, Zhengzhi Wu, Meiqun Cao
Neurodegenerative diseases are among the most serious health problems affecting millions of people worldwide. Such diseases are characterized by a progressive degeneration and / or death of neurons in the central nervous system. Currently, there are no therapeutic approaches to cure or even halt the progression of neurodegenerative diseases. During the last two decades, much attention has been paid to the neuroprotective and anti-neurodegenerative activities of compounds isolated from natural products with high efficacy and low toxicity...
April 19, 2018: Current Neuropharmacology
https://www.readbyqxmd.com/read/29676205/effects-of-peptidyl-prolyl-isomerase-1-depletion-in-animal-models-of-prion-diseases
#3
Giuseppe Legname, Tommaso Virgilio, Edoardo Bistaffa, Chiara Maria Giulia De Luca, Marcella Catania, Paola Zago, Elisa Isopi, Ilaria Campagnani, Fabrizio Tagliavini, Giorgio Giaccone, Fabio Moda
Pin1 is a peptidyl-prolyl isomerase that induces the cis-trans conversion of specific Ser/Thr-Pro peptide bonds in phosphorylated proteins, leading to conformational changes through which Pin1 regulates protein stability and activity. Since down-regulation of Pin1 has been described in several neurodegenerative disorders, including Alzheimer's Disease (AD), Parkinson's Disease (PD) and Huntington's Disease (HD), we investigated its potential role in prion diseases. Animals generated on wild-type (Pin1+/+ ), hemizygous (Pin1+/- ) or knock-out (Pin1-/- ) background for Pin1 were experimentally infected with RML prions...
April 20, 2018: Prion
https://www.readbyqxmd.com/read/29674695/how-high-energy-fluxes-may-affect-rayleigh-taylor-instability-growth-in-young-supernova-remnants
#4
C C Kuranz, H-S Park, C M Huntington, A R Miles, B A Remington, T Plewa, M R Trantham, H F Robey, D Shvarts, A Shimony, K Raman, S MacLaren, W C Wan, F W Doss, J Kline, K A Flippo, G Malamud, T A Handy, S Prisbrey, C M Krauland, S R Klein, E C Harding, R Wallace, M J Grosskopf, D C Marion, D Kalantar, E Giraldez, R P Drake
Energy-transport effects can alter the structure that develops as a supernova evolves into a supernova remnant. The Rayleigh-Taylor instability is thought to produce structure at the interface between the stellar ejecta and the circumstellar matter, based on simple models and hydrodynamic simulations. Here we report experimental results from the National Ignition Facility to explore how large energy fluxes, which are present in supernovae, affect this structure. We observed a reduction in Rayleigh-Taylor growth...
April 19, 2018: Nature Communications
https://www.readbyqxmd.com/read/29671352/huntington-s-disease-novel-therapeutic-perspectives-hanging-in-the-balance
#5
Ana Saavedra, Gerardo García-Díaz Barriga, Esther Pérez-Navarro, Jordi Alberch
Huntington's disease (HD), an autosomal dominant neurodegenerative disorder caused by an expansion of CAG repeats in the huntingtin gene, has long been characterized by the presence of motor symptoms due to the loss of striatal projection neurons. Cognitive dysfunction and neuropsychiatric symptoms are also present and they occur in the absence of cell death in most mouse models, pointing to neuronal dysfunction and abnormal synaptic plasticity as causative mechanisms. Areas covered: Here, we focus on those common mechanisms altered by the presence of mutant huntingtin affecting corticostriatal and hippocampal function as therapeutic targets that could prove beneficial to ameliorate both cognitive and motor function in HD...
April 19, 2018: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/29670796/huntington-s-disease-in-a-patient-misdiagnosed-as-conversion-disorder
#6
João Machado Nogueira, Ana Margarida Franco, Susana Mendes, Anabela Valadas, Cristina Semedo, Gustavo Jesus
Huntington's disease (HD) is an inherited, progressive, and neurodegenerative neuropsychiatric disorder caused by the expansion of cytosine-adenine-guanine (CAG) trinucleotide in Interested Transcript (IT) 15 gene on chromosome 4. This pathology typically presents in individuals aged between 30 and 50 years and the age of onset is inversely correlated with the length of the CAG repeat expansion. It is characterized by chorea, cognitive deficits, and psychiatric symptoms. Usually the psychiatric disorders precede motor and cognitive impairment, Major Depressive Disorder and anxiety disorders being the most common presentations...
2018: Case Reports in Psychiatry
https://www.readbyqxmd.com/read/29668904/a-whole-brain-longitudinal-study-in-the-yac128-mouse-model-of-huntington-s-disease-shows-distinct-trajectories-of-neurochemical-structural-connectivity-and-volumetric-changes
#7
Lorena I Petrella, João M Castelhano, Mario Ribeiro, José V Sereno, Sónia I Gonçalves, Mário N Laço, Michael R Hayden, Ana C Rego, Miguel Castelo-Branco
Huntington's disease (HD) is a neurodegenerative disorder causing cognitive and motor impairments, evolving to death within 15-20 years after symptom onset. We previously established a mouse model with the entire human HD gene containing 128 CAG repeats (YAC128) which accurately recapitulates the natural history of the human disease. Defined time points in this natural history enable the understanding of longitudinal trajectories from the neurochemical and structural points of view using non-invasive high-resolution multimodal imaging...
April 16, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29667946/deutetrabenazine-austedo-for-huntington-s-chorea-and-tardive-dyskinesia
#8
(no author information available yet)
No abstract text is available yet for this article.
April 23, 2018: Medical Letter on Drugs and Therapeutics
https://www.readbyqxmd.com/read/29666090/rare-case-of-chorea-hyperglycaemia-basal-ganglia-c-h-bg-syndrome
#9
Sajjad Ahmad, Priya Mohan Babu, Lavanya Shenbagaraj, Lindsay George
An 83-year-old woman presented with acute-onset haemichorea and haemiballism particularly affecting the left side of the body. She was known to have type 2 diabetes, which was poorly controlled with sitagliptin. She was hyperglycaemic but not ketotic or acidotic. After she was started on insulin and good glycaemic control was achieved, her abnormal movements dramatically improved. MRI of the brain showed a T1-weighted hyperintense lesion on the right basal ganglia, which is typical of chorea-hyperglycaemia-basal ganglia syndrome...
April 17, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29665395/label-free-colorimetric-sensor-for-sensitive-detection-of-choline-based-on-dnazyme-choline-oxidase-coupling
#10
Nasrin Nikzad, Zahra Karami
Changes in choline levels can be associated with diseases such as Alzheimer, Parkinson, Huntington, fatty liver, interstitial lung abnormalities, autism and so on. Therefore, quantitative determination of choline is important in biological and clinical analysis. So far, several methods have been investigated for measuring choline in the body fluids, each of which has disadvantages such as the need for specialist ability, complexity, and high cost. For this purpose, a facile and sensitive colorimetric biosensor based on DNAzyme-choline oxidase coupling used for the determination of choline...
April 14, 2018: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/29664151/the-receptor-for-advanced-glycation-endproducts-is-a-mediator-of-toxicity-by-iapp-and-other-proteotoxic-aggregates-establishing-and-exploiting-common-ground-for-novel-amyloidosis-therapies
#11
REVIEW
Andisheh Abedini, Julia Derk, Ann Marie Schmidt
Proteotoxicity plays a key role in many devastating human disorders, including Alzheimer's, Huntington's and Parkinson's diseases; type 2 diabetes; systemic amyloidosis; and cardiac dysfunction, to name a few. The cellular mechanisms of proteotoxicity in these disorders have been the focus of considerable research, but their role in prevalent and morbid disorders, such as diabetes, is less appreciated. There is a large body of literature on the impact of glucotoxicity and lipotoxicity on insulin-producing pancreatic β-cells, and there is increasing recognition that proteotoxicty plays a key role...
April 17, 2018: Protein Science: a Publication of the Protein Society
https://www.readbyqxmd.com/read/29663942/longitudinal-anthropometric-assessment-of-rhesus-macaque-macaca-mulatta-model-of-huntington-disease
#12
Carissa E Hunter, Alvince L Pongos, Tim Y Chi, Christa Payne, Fawn C Stroud, Anthony W S Chan
The neurodegeneration associated with Huntington disease (HD) leads to the onset of motor and cognitive impairment and their advancement with increased age in humans. In children at risk for HD, body measurement growth abnormalities include a reduction in BMI, weight, height, and head circumference. The transgenic HD NHP model was first reported in 2008, and progressive decline in cognitive behaviors and motor impairment have been reported. This study focuses on longitudinal body measurements in HD macaques from infancy through adulthood...
April 2, 2018: Comparative Medicine
https://www.readbyqxmd.com/read/29660633/objective-assessment-of-gait-and-posture-in-premanifest-and-manifest-huntington-disease-a-multi-center-study
#13
Heike Beckmann, Stefan Bohlen, Carsten Saft, Rainer Hoffmann, Joachim Gerss, Lisa Muratori, E Bernd Ringelstein, G Bernhard Landwehrmeyer, Ralf Reilmann
BACKGROUND: Deficits in posture and gait are known to contribute to the complex motor phenotype of Huntington disease (HD). Objective and quantitative measures of posture and gait provided by posturography and GAITRite® assessments may supplement categorical rating scales such as the UHDRS-TMS and increase power and sensitivity of clinical trials. OBJECTIVES: To investigate whether posturography and GAITRite® measures reveal (1) changes in manifest or premanifest HD mutation-carriers, (2) a correlation to the UHDRS-TMS and functional measures in manifest HD, and (3) a correlation to the disease-burden-score (based on CAG-repeat-length and age)...
April 7, 2018: Gait & Posture
https://www.readbyqxmd.com/read/29659047/the-changing-face-of-adult-posttransplant-lymphoproliferative-disorder-changes-in-histology-between-1999-2013
#14
Donald E Tsai, Stephen Bagley, Ran Reshef, Abraham Shaked, Roy D Bloom, Vivek Ahya, Lee Goldberg, Albert Chung, Fotini Debonera, Stephen J Schuster, Scott F Huntington
Posttransplant lymphoproliferative disorder (PTLD) typically presents with either polymorphic or monomorphic histology. While both are the end result of immunosuppressive therapies, their origins are felt to be different with different prognoses and responsiveness to therapy, resulting in two different malignancies. We attempted to confirm reports suggesting that the relative frequency of these two histologies is shifting over time. We analyzed 3040 adult PTLD cases in the UNOS OPTN database from 1999 to 2013...
April 16, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29657306/hypoxia-induced-microrna-210-targets-neurodegenerative-pathways
#15
Michelle E Watts, Sarah M Williams, Jess Nithianantharajah, Charles Claudianos
Hypoxia-regulated microRNA-210 (miR-210) is a highly conserved microRNA, known to regulate various processes under hypoxic conditions. Previously we found that miR-210 is also involved in honeybee learning and memory, raising the questions of how neural activity may induce hypoxia-regulated genes and how miR-210 may regulate plasticity in more complex mammalian systems. Using a pull-down approach, we identified 620 unique target genes of miR-210 in humans, among which there was a significant enrichment of age-related neurodegenerative pathways, including Huntington's, Alzheimer's, and Parkinson's diseases...
March 27, 2018: Non-Coding RNA
https://www.readbyqxmd.com/read/29652574/transcriptomic-biomarkers-for-huntington-s-disease-are-gene-expression-signatures-in-whole-blood-reliable-biomarkers
#16
Maja Zadel, Aleš Maver, Anja Kovanda, Borut Peterlin
Huntington's disease (HD) is a severe neurodegenerative disorder manifesting as progressive impairment of motor function, cognitive decline, psychiatric symptoms, and immunological and endocrine dysfunction. We explored the consistency of blood transcriptomic biomarkers in HD based on a novel Slovene patient cohort and expert review of previous studies. HumanHT-12 v4 BeadChip microarrays were performed on the whole blood samples of a cohort of 23 HD mutation carriers and 23 controls to identify differentially expressed (DE) transcripts...
April 2018: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/29651271/genetics-modulate-gray-matter-variation-beyond-disease-burden-in-prodromal-huntington-s-disease
#17
Jingyu Liu, Jennifer Ciarochi, Vince D Calhoun, Jane S Paulsen, H Jeremy Bockholt, Hans J Johnson, Jeffrey D Long, Dongdong Lin, Flor A Espinoza, Maria B Misiura, Arvind Caprihan, Jessica A Turner
Huntington's disease (HD) is a neurodegenerative disorder caused by an expansion mutation of the cytosine-adenine-guanine (CAG) trinucleotide in the HTT gene. Decline in cognitive and motor functioning during the prodromal phase has been reported, and understanding genetic influences on prodromal disease progression beyond CAG will benefit intervention therapies. From a prodromal HD cohort ( N  = 715), we extracted gray matter (GM) components through independent component analysis and tested them for associations with cognitive and motor functioning that cannot be accounted for by CAG-induced disease burden (cumulative effects of CAG expansion and age)...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29650257/neuropeptide-y-mitigates-er-stress-induced-neuronal-cell-death-by-activating-the-pi3k-xbp1-pathway
#18
Do Yeon Lee, Seung Hyun Hong, Bokyung Kim, Dong-Seok Lee, Kweon Yu, Kyu-Sun Lee
The unfolded protein response (UPR) is an evolutionarily conserved adaptive reaction that increases cell survival under endoplasmic reticulum (ER) stress conditions. ER stress-associated neuronal cell death pathways play roles in the pathogenesis of neurodegenerative diseases, including Alzheimer's, Parkinson's, and Huntington's disease. Neuropeptide Y (NPY) has an important role in neuroprotection against neurodegenerative diseases. In this study, we investigated whether NPY has a protective role in ER stress-induced neuronal cell death in SK-N-SH human neuroblastoma cells...
April 6, 2018: European Journal of Cell Biology
https://www.readbyqxmd.com/read/29643462/environment-dependent-striatal-gene-expression-in-the-bachd-rat-model-for-huntington-disease
#19
Arianna Novati, Thomas Hentrich, Zinah Wassouf, Jonasz J Weber, Libo Yu-Taeger, Nicole Déglon, Huu Phuc Nguyen, Julia M Schulze-Hentrich
Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a mutation in the huntingtin (HTT) gene which results in progressive neurodegeneration in the striatum, cortex, and eventually most brain areas. Despite being a monogenic disorder, environmental factors influence HD characteristics. Both human and mouse studies suggest that mutant HTT (mHTT) leads to gene expression changes that harbor potential to be modulated by the environment. Yet, the underlying mechanisms integrating environmental cues into the gene regulatory program have remained largely unclear...
April 11, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29643420/publisher-correction-alteration-in-fluidity-of-cell-plasma-membrane-in-huntington-disease-revealed-by-spectral-phasor-analysis
#20
Sara Sameni, Leonel Malacrida, Zhiqun Tan, Michelle A Digman
A correction to this article has been published and is linked from the HTML and PDF versions of this paper. The error has been fixed in the paper.
April 11, 2018: Scientific Reports
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