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https://www.readbyqxmd.com/read/28926091/functional-role-of-mesenchymal-stem-cells-in-the-treatment-of-chronic-neurodegenerative-diseases
#1
Debora Lo Furno, Giuliana Mannino, Rosario Giuffrida
Mesenchymal stem cells (MSCs) can differentiate into not only cells of mesodermal lineages, but also into endodermal and ectodermal derived elements, including neurons and glial cells. For this reason, MSCs have been extensively investigated to develop cell-based therapeutic strategies, especially in pathologies whose pharmacological treatments give poor results, if any. As in the case of irreversible neurological disorders characterized by progressive neuronal death, in which behavioral and cognitive functions of patients inexorably decline as the disease progresses...
September 19, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28923312/autophagy-its-mechanisms-and-regulation-implications-in-neurodegenerative-diseases
#2
REVIEW
Milad Moloudizargari, Mohammad Hossein Asghari, Emad Ghobadi, Marjan Fallah, Shima Rasouli, Mohammad Abdollahi
Autophagy is a major regulatory cellular mechanism which gives the cell an ability to cope with some of the destructive events that normally occur within a metabolically living cell. This is done by maintaining the cellular homeostasis, clearance of damaged organelles and proteins and recycling necessary molecules like amino acids and fatty acids. There is a wide array of factors that influence autophagy in the state of health and disease. Disruption of these mechanisms may not only give rise to several autophagy-related disease, but also it can occur as the result of intracellular changes induced during disease pathogenesis causing exacerbation of the disease...
September 15, 2017: Ageing Research Reviews
https://www.readbyqxmd.com/read/28923065/the-heat-shock-response-in-neurons-and-astroglia-and-its-role-in-neurodegenerative-diseases
#3
REVIEW
Rebecca San Gil, Lezanne Ooi, Justin J Yerbury, Heath Ecroyd
Protein inclusions are a predominant molecular pathology found in numerous neurodegenerative diseases, including amyotrophic lateral sclerosis and Huntington's disease. Protein inclusions form in discrete areas of the brain characteristic to the type of neurodegenerative disease, and coincide with the death of neurons in that region (e.g. spinal cord motor neurons in amyotrophic lateral sclerosis). This suggests that the process of protein misfolding leading to inclusion formation is neurotoxic, and that cell-autonomous and non-cell autonomous mechanisms that maintain protein homeostasis (proteostasis) can, at times, be insufficient to prevent protein inclusion formation in the central nervous system...
September 18, 2017: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/28922164/analysis-of-participant-withdrawal-in-huntington-disease-clinical-trials
#4
Haruhiko Banno, Kelly L Andrzejewski, Michael P McDermott, Alyssa Murphy, Madhurima Majumder, Elisabeth A de Blieck, Peggy Auinger, Merit E Cudkowicz, Nazem Atassi
No abstract text is available yet for this article.
September 14, 2017: Journal of Huntington's Disease
https://www.readbyqxmd.com/read/28920889/therapies-targeting-dna-and-rna-in-huntington-s-disease
#5
REVIEW
Edward J Wild, Sarah J Tabrizi
No disease-slowing treatment exists for Huntington's disease, but its monogenic inheritance makes it an appealing candidate for the development of therapies targeting processes close to its genetic cause. Huntington's disease is caused by CAG repeat expansions in the HTT gene, which encodes the huntingtin protein; development of therapies to target HTT transcription and the translation of its mRNA is therefore an area of intense investigation. Huntingtin-lowering strategies include antisense oligonucleotides and RNA interference targeting mRNA, and zinc finger transcriptional repressors and CRISPR-Cas9 methods aiming to reduce transcription by targeting DNA...
October 2017: Lancet Neurology
https://www.readbyqxmd.com/read/28920551/a-comprehensive-in-silico-analysis-of-huntingtin-and-its-interactome
#6
Valentina Brandi, Valentina Di Lella, Maria Marino, Paolo Ascenzi, Fabio Polticelli
A polyglutamine expansion of the N-terminal region of huntingtin (Htt) causes Huntington's disease (HD), a severe neurodegenerative disorder. Htt huge multidomain structure, the presence of disordered regions, and the lack of sequence homologs of known structure, so far prevented structural studies of Htt, making the study of its structure-function relationships very difficult. In this work, the presence and location of five Htt ordered domains (named from Hunt1 to Hunt5) has been detected and the structure of these domains has been predicted for the first time using a combined threading/ab initio modelling approach...
September 18, 2017: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/28920088/metabolic-and-transcriptomic-analysis-of-huntington-s-disease-model-reveal-changes-in-intracellular-glucose-levels-and-related-genes
#7
Gepoliano Chaves, Rıfat Emrah Özel, Namrata V Rao, Hana Hadiprodjo, Yvonne Da Costa, Zachary Tokuno, Nader Pourmand
Huntington's Disease (HD) is a neurodegenerative disorder caused by an expansion in a CAG-tri-nucleotide repeat that introduces a poly-glutamine stretch into the huntingtin protein (mHTT). Mutant huntingtin (mHTT) has been associated with several phenotypes including mood disorders and depression. Additionally, HD patients are known to be more susceptible to type II diabetes mellitus (T2DM), and HD mice model develops diabetes. However, the mechanism and pathways that link Huntington's disease and diabetes have not been well established...
August 2017: Heliyon
https://www.readbyqxmd.com/read/28920068/tetrabenazine-versus-deutetrabenazine-for-huntington-s-disease-twins-or-distant-cousins
#8
Filipe B Rodrigues, Gonçalo S Duarte, João Costa, Joaquim J Ferreira, Edward J Wild
BACKGROUND: Tetrabenazine is the only US Food and Drug Administration-approved drug for Huntington's disease, and deutetrabenazine was recently tested against placebo. A switching-trial from tetrabenazine to deutetrabenazine is underway, but no head-to-head, blinded, randomized controlled trial is planned. Using meta-analytical methodology, the authors compared these molecules. METHODS: RCTs comparing tetrabenazine or deutetrabenazine with placebo in Huntington's disease were searched...
July 2017: Movement Disorders Clinical Practice
https://www.readbyqxmd.com/read/28917260/roles-of-sigma-1-receptors-on-mitochondrial-functions-relevant-to-neurodegenerative-diseases
#9
REVIEW
Tzu-Yu Weng, Shang-Yi Anne Tsai, Tsung-Ping Su
The sigma-1 receptor (Sig-1R) is a chaperone that resides mainly at the mitochondrion-associated endoplasmic reticulum (ER) membrane (called the MAMs) and acts as a dynamic pluripotent modulator in living systems. At the MAM, the Sig-1R is known to play a role in regulating the Ca(2+) signaling between ER and mitochondria and in maintaining the structural integrity of the MAM. The MAM serves as bridges between ER and mitochondria regulating multiple functions such as Ca(2+) transfer, energy exchange, lipid synthesis and transports, and protein folding that are pivotal to cell survival and defense...
September 16, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28916295/utilization-of-hospice-services-in-a-population-of-patients-with-huntington-s-disease
#10
Margaret O Johnson, Samuel Frank, Matthew Mendlik, David Casarett
CONTEXT: Although the early and middle stages of Huntington's Disease (HD) and its complications have been well described, less is known about the course of late-stage illness. In particular, little is known about the population of patients who enroll in hospice. OBJECTIVES: Our goal is to describe the characteristics of patients with HD who enrolled in hospice. METHODS: Retrospective cohort study of electronic medical record data from 12 not-for-profit hospices in the United States from 2008 to 2012...
September 12, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/28912682/extracellular-vesicles-in-brain-tumors-and-neurodegenerative-diseases
#11
REVIEW
Federica Ciregia, Andrea Urbani, Giuseppe Palmisano
Extracellular vesicles (EVs) can be classified into apoptotic bodies, microvesicles (MVs), and exosomes, based on their origin or size. Exosomes are the smallest and best characterized vesicles which derived from the endosomal system. These vesicles are released from many different cell types including neuronal cells and their functions in the nervous system are investigated. They have been proposed as novel means for intercellular communication, which takes part not only to the normal neuronal physiology but also to the transmission of pathogenic proteins...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28906031/design-optimization-for-clinical-trials-in-early-stage-manifest-huntington-s-disease
#12
Chris Frost, Amy Mulick, Rachael I Scahill, Gail Owen, Elizabeth Aylward, Blair R Leavitt, Alexandra Durr, Raymund A C Roos, Beth Borowsky, Julie C Stout, Ralf Reilmann, Douglas R Langbehn, Sarah J Tabrizi, Cristina Sampaio
OBJECTIVES: The purpose of this study was to inform the design of randomized clinical trials in early-stage manifest Huntington's disease through analysis of longitudinal data from TRACK-Huntington's Disease (TRACK-HD), a multicenter observational study. METHODS: We compute sample sizes required for trials with candidate clinical, functional, and imaging outcomes, whose aims are to reduce rates of change. The calculations use a 2-stage approach: first using linear mixed models to estimate mean rates of change and components of variability from TRACK-HD data and second using these to predict sample sizes for a range of trial designs...
September 14, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28904554/plants-derived-neuroprotective-agents-cutting-the-cycle-of-cell-death-through-multiple-mechanisms
#13
REVIEW
Taiwo Olayemi Elufioye, Tomayo Ireti Berida, Solomon Habtemariam
Neuroprotection is the preservation of the structure and function of neurons from insults arising from cellular injuries induced by a variety of agents or neurodegenerative diseases (NDs). The various NDs including Alzheimer's, Parkinson's, and Huntington's diseases as well as amyotropic lateral sclerosis affect millions of people around the world with the main risk factor being advancing age. Each of these diseases affects specific neurons and/or regions in the brain and involves characteristic pathological and molecular features...
2017: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/28902882/risk-of-myeloid-neoplasms-after-radiotherapy-among-older-women-with-localized-breast-cancer-a-population-based-study
#14
Amer M Zeidan, Jessica B Long, Rong Wang, Xin Hu, James B Yu, Scott F Huntington, Gregory A Abel, Sarah S Mougalian, Nikolai A Podoltsev, Steven D Gore, Cary P Gross, Xiaomei Ma, Amy J Davidoff
BACKGROUND: There are inconsistent and limited data regarding the risk of myeloid neoplasms (MN) among breast cancer survivors who received radiotherapy (RT) in the absence of chemotherapy. Concern about subsequent MN might influence the decision to use adjuvant RT for women with localized disease. As patients with therapy-related MN have generally poor outcomes, the presumption of subsequent MN being therapy-related could affect treatment recommendations. METHODS: We used the Surveillance, Epidemiology, and End Results (SEER)-Medicare linked database to study older women with in-situ or stage 1-3 breast cancer diagnosed 2001-2009 who received surgery...
2017: PloS One
https://www.readbyqxmd.com/read/28901130/sequential-feature-density-doubling-for-ultraviolet-plasmonics
#15
Michael P Knudson, Alexander J Hryn, Mark D Huntington, Teri W Odom
Production of nanostructures with sub-200-nm periodicities over cm2-scale areas is challenging using standard patterning approaches. This paper demonstrates a scalable technique for feature-density doubling that can generate nanopatterned lines with periodicities down to 100 nm covering >3 cm2. We developed a process based on controlled wet over-etching of atomic-layered deposited alumina to tune feature sizes down to several nm in the alumina masking layer. These features transferred into silicon served as masters for template-striping aluminum nano-gratings with three different periodicities...
September 13, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28900136/differential-effects-of-soluble-and-aggregating-polyq-proteins-on-cytotoxicity-and-type-1-myosin-dependent-endocytosis-in-yeast
#16
Lisa L Berglund, Xinxin Hao, Beidong Liu, Julie Grantham, Thomas Nyström
Huntington's disease develops when the polyglutamine (polyQ) repeat in the Huntingtin (Htt) protein is expanded to over 35 glutamines rendering it aggregation-prone. Here, using Htt exon-1 as a polyQ model protein in a genome-wide screen in yeast, we show that the normal and soluble Htt exon-1 is toxic in cells with defects in type-1 myosin-dependent endocytosis. The toxicity of Htt is linked to physical interactions with type-1 myosins, which occur via the Htt proline-rich region, leading to a reduction in actin patch polarization and clathrin-dependent endocytosis...
September 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28900094/-protein-protein-interactions-of-huntingtin-in-the-hippocampus
#17
A L Proskura, S O Vechkapova, T A Zapara, A S Ratushniak
Huntingtin (HTT) occurs in the neuronal cytoplasm and can interact with structural elements of synapses. Huntington's disease (HD) results from pathological expansion of a polyglutamine stretch in the HTT molecule, being probably associated with aberrant protein-protein interactions. The pathogenetic mechanism is still incompletely understood. Alterations of the synaptic structure and plasticity in the hippocampus are observed in early HD. The objective of the study was to theoretically evaluate the HTT contribution to changes in synaptic plasticity by integrating the available experimental data...
July 2017: Molekuliarnaia Biologiia
https://www.readbyqxmd.com/read/28899183/engaging-with-climate-change-psychoanalytic-and-interdisciplinary-perspectives-and-laudato-si-on-care-for-our-common-home-engaging-with-climate-change-psychoanalytic-and-interdisciplinary-perspectives-edited-by-weintrobe-sally-new-york-routledge-2013-xxii-254
#18
https://www.readbyqxmd.com/read/28894988/how-tandem-gait-stumbled-into-the-neurological-exam-a-review
#19
Jason Margolesky, Carlos Singer
Tandem gait testing is an integral part of the neurological exam. It is informative in a wide variety of disorders ranging from cerebellar disease to vestibular and peripheral neuropathies, parkinsonism, and other neurodegenerative conditions. We discuss the history and development of tandem gait testing as well as its technique, utility, and limitations in the assessment of neurological conditions. Tandem gait has emerged as a tool in the assessment of cerebellar disease, Huntington disease, idiopathic Parkinson's disease, atypical parkinsonism, peripheral neuropathies, and vestibulopathies...
September 11, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28893312/the-validity-and-reliability-of-the-four-square-step-test-in-different-adult-populations-a-systematic-review
#20
Martha Moore, Karen Barker
BACKGROUND: The four square step test (FSST) was first validated in healthy older adults to provide a measure of dynamic standing balance and mobility. The FSST has since been used in a variety of patient populations. The purpose of this systematic review is to determine the validity and reliability of the FSST in these different adult patient populations. METHODS: The literature search was conducted to highlight all the studies that measured validity and reliability of the FSST...
September 11, 2017: Systematic Reviews
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