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Related ArticlesProtein Misfolding and ER Stress in Huntington's Disease. Front Mol Biosci. 2019;6:20 Authors: Shacham T, Sharma N, Lederkremer GZ Abstract Increasing evidence in recent years indicates that protein misfolding and aggregation, leading to ER stress, are central factors of pathogenicity in neurodegenerative diseases. This is particularly true in Huntington's ... read more

Related ArticlesImproving follow up after predictive testing in Huntington's disease: evaluating a genetic counselling narrative group session. J Community Genet. 2019 Apr 18;: Authors: Stopford C, Ferrer-Duch M, Moldovan R, MacLeod R Abstract Recently updated Huntington's disease (HD) predictive testing guidelines emphasise clinicians' responsibility to facilitate emotional support following testing, ... read more

Related ArticlesFelodipine induces autophagy in mouse brains with pharmacokinetics amenable to repurposing. Nat Commun. 2019 Apr 18;10(1):1817 Authors: Siddiqi FH, Menzies FM, Lopez A, Stamatakou E, Karabiyik C, Ureshino R, Ricketts T, Jimenez-Sanchez M, Esteban MA, Lai L, Tortorella MD, Luo Z, Liu H, Metzakopian E, Fernandes HJR, Bassett A, ... read more

Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription. Elife. 2019 Apr 17;8: Authors: Gao R, Chakraborty A, Geater C, Pradhan S, Gordon KL, Snowden J, Yuan S, Dickey AS, Choudhary S, Ashizawa T, Ellerby LM, La Spada AR, Thompson LM, Hazra TK, Sarkar PS Abstract How huntingtin ... read more

Depression in Neurodegenerative Diseases: Common Mechanisms and Current Treatment Options. Neurosci Biobehav Rev. 2019 Apr 14;: Authors: Galts CPC, Bettio LEB, Jewett DC, Yang CC, Brocardo PS, Rodrigues ALS, Thacker JS, Gil-Mohapel J Abstract Major depressive disorder (MDD) is a highly prevalent psychiatric disorder and a major cause of disability ... read more

Related ArticlesModular probes for enriching and detecting complex nucleic acid sequences. Nat Chem. 2017 12;9(12):1222-1228 Authors: Wang JS, Yan YH, Zhang DY Abstract Complex DNA sequences are difficult to detect and profile, but are important contributors to human health and disease. Existing hybridization probes lack the capability to ... read more

Related ArticlesIdentification of key pathways and genes in nasopharyngeal carcinoma using bioinformatics analysis. Oncol Lett. 2019 May;17(5):4683-4694 Authors: Zhu HM, Fei Q, Qian LX, Liu BL, He X, Yin L Abstract Nasopharyngeal carcinoma (NPC) is one of the most common malignancies in the head and neck. The aim of the ... read more

The O-GlcNAc modification protects against protein misfolding and aggregation in neurodegenerative disease. ACS Chem Neurosci. 2019 Apr 15;: Authors: Ryan P, Xu M, Davey AK, Danon JJ, Mellick GD, Kassiou M, Rudrawar S Abstract Post-translational modifications (PTM) of proteins are becoming the focus of intense research due to their implications ... read more

AAV5-miHTT Gene Therapy Demonstrates Sustained Huntingtin Lowering and Functional Improvement in Huntington Disease Mouse Models. Mol Ther Methods Clin Dev. 2019 Jun 14;13:334-343 Authors: Spronck EA, Brouwers CC, Vallès A, de Haan M, Petry H, van Deventer SJ, Konstantinova P, Evers MM Abstract Huntington disease (HD) is a fatal neurodegenerative ... read more

Widespread Striatal Delivery of GDNF from Encapsulated Cells Prevents the Anatomical and Functional Consequences of Excitotoxicity. Neural Plast. 2019;2019:6286197 Authors: Emerich DF, Kordower JH, Chu Y, Thanos C, Bintz B, Paolone G, Wahlberg LU Abstract Methods: Human ARPE-19 cells engineered to secrete high levels of the glial cell line-derived neurotrophic ... read more

Related ArticlesDual Role of Ribosome-Binding Domain of NAC as a Potent Suppressor of Protein Aggregation and Aging-Related Proteinopathies. Mol Cell. 2019 Apr 08;: Authors: Shen K, Gamerdinger M, Chan R, Gense K, Martin EM, Sachs N, Knight PD, Schlömer R, Calabrese AN, Stewart KL, Leiendecker L, Baghel A, Radford SE, ... read more

Related ArticlesThe role of the protein-RNA recognition code in neurodegeneration. Cell Mol Life Sci. 2019 Apr 12;: Authors: Nahalka J Abstract MicroRNAs are small endogenous RNAs that pair and bind to sites on mRNAs to direct post-transcriptional repression. However, there is a possibility that microRNAs directly influence protein structure and ... read more

Related ArticlesBrain structure in juvenile-onset Huntington disease. Neurology. 2019 Apr 10;: Authors: Tereshchenko A, Magnotta V, Epping E, Mathews K, Espe-Pfeifer P, Martin E, Dawson J, Duan W, Nopoulos P Abstract OBJECTIVE: To assess brain morphometry in a sample of patients with juvenile-onset Huntington disease (JOHD) and several mouse models ... read more

Related ArticlesJeff Carroll. Lancet Neurol. 2017 Feb;16(2):108 Authors: PMID: 28102149 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesA Novel Ecological Approach Reveals Early Executive Function Impairments in Huntington's Disease. Front Psychol. 2019;10:585 Authors: Júlio F, Ribeiro MJ, Patrício M, Malhão A, Pedrosa F, Gonçalves H, Simões M, van Asselen M, Simões MR, Castelo-Branco M, Januário C Abstract Introduction: Impairments in executive functions are common in neurogenetic ... read more

Related ArticlesYeast Models for the Study of Amyloid-Associated Disorders and Development of Future Therapy. Front Mol Biosci. 2019;6:15 Authors: Rencus-Lazar S, DeRowe Y, Adsi H, Gazit E, Laor D Abstract First described almost two decades ago, the pioneering yeast models of neurodegenerative disorders, including Alzheimer's, Parkinson's, and Huntington's diseases, have ... read more

Small molecule targeting of RNA structures in neurological disorders. Ann N Y Acad Sci. 2019 Apr 09;: Authors: Angelbello AJ, Chen JL, Disney MD Abstract Aberrant RNA structure and function operate in neurological disease progression and severity. As RNA contributes to disease pathology in a complex fashion, that is, via ... read more

Related ArticlesModulation of mTOR and CREB pathways following mGluR5 blockade contribute to improved Huntington's pathology in zQ175 mice. Mol Brain. 2019 04 08;12(1):35 Authors: Abd-Elrahman KS, Ferguson SSG Abstract Huntington's disease (HD) is a neurodegenerative disorder caused by a genetic abnormality in the huntingtin gene that leads to a polyglutamine ... read more

Impact of sex differences and gender specificity on behavioral characteristics and pathophysiology of neurodegenerative disorders. Neurosci Biobehav Rev. 2019 Apr 05;: Authors: Ullah MF, Ahmad A, Bhat SH, Abu-Duhier FM, Barreto GE, Ashraf GM Abstract The impact of neurodegenerative disorders in humans has multiple consequences because of the progressive decline ... read more

Opportunities and challenges for the use of induced pluripotent stem cells in modelling neurodegenerative disease. Open Biol. 2019 Jan 31;9(1):180177 Authors: Wu YY, Chiu FL, Yeh CS, Kuo HC Abstract Adult-onset neurodegenerative diseases are among the most difficult human health conditions to model for drug development. Most genetic or toxin-induced ... read more

Striatal Cholesterol Precursors Are Altered with Age in Female Huntington's Disease Model Mice. J Huntingtons Dis. 2019 Apr 03;: Authors: Pfalzer AC, Wages PA, Porter NA, Bowman AB Abstract BACKGROUND: Cholesterol is necessary for proper neurodevelopment and neuronal health. The brain relies on neural and astrocytic de novo cholesterol synthesis. ... read more

Related ArticlesRetinal single-layer analysis with optical coherence tomography shows inner retinal layer thinning in Huntington's disease as a potential biomarker. Int Ophthalmol. 2019 Mar;39(3):611-621 Authors: Gulmez Sevim D, Unlu M, Gultekin M, Karaca C Abstract PURPOSE: There have been ongoing clinical trials of therapeutic agents in Huntington's disease (HD) ... read more

Related ArticlesExpression of Concern: Kumar P, Kalonia H and Kumar A. 2011, Role of LOX/COX pathways in 3-nitropropionic acid-induced Huntington's disease-like symptoms in rats: Protective effect of licofelone. Brit J Pharmacol 164: 644-654. doi: 10.1111/j.1476-5381.2011.01418.x. Br J Pharmacol. 2019 Apr;176(8):1170 Authors: Abstract This expression of concern is for the ... read more

PDE10A mutations help to unwrap the neurobiology of hyperkinetic disorders. Cell Signal. 2019 Apr 02;: Authors: Whiteley EL, Tejeda GS, Baillie GS, Brandon NJ Abstract The dual-specific cAMP/cGMP phosphodiesterase PDE10A is exclusively localised to regions of the brain and specific cell types that control crucial brain circuits and behaviours. The ... read more

Related ArticlesEffects of Curcumin on Microglial Cells. Neurotox Res. 2019 Apr 04;: Authors: Ghasemi F, Bagheri H, Barreto GE, Read MI, Sahebkar A Abstract Microglia are innate immune system cells which reside in the central nervous system (CNS). Resting microglia regulate the homeostasis of the CNS via phagocytic activity to ... read more

Related ArticlesThe Effects of Dual-Task Cognitive Interference and Environmental Challenges on Balance in Huntington's Disease. Mov Disord Clin Pract. 2019 Mar;6(3):202-212 Authors: Purcell NL, Goldman JG, Ouyang B, Bernard B, O'Keefe JA Abstract Background: Huntington's disease (HD) is characterized by chorea, balance and gait impairments, and cognitive deficits, which increase ... read more

Related ArticlesSilencing of the Mutant Huntingtin Gene through CRISPR-Cas9 Improves the Mitochondrial Biomarkers in an In Vitro Model of Huntington's Disease. Cell Transplant. 2019 Apr 04;:963689719840662 Authors: Dunbar GL, Koneru S, Kolli N, Sandstrom M, Maiti P, Rossignol J Abstract During the 25-year history of the American Society for Neural ... read more

Attending to Total Pain in Juvenile Huntington Disease: A Case Report Informed by Narrative Review of the Literature. J Palliat Care. 2019 Apr 05;:825859719835560 Authors: Kendrick LM, Hudgell D, Hellman A, Weaver MS Abstract OBJECTIVES:: To consider the impact of juvenile Huntington disease (JHD) from a biomedical, symptom burden, and ... read more

Related ArticlesRole of Plant Derived Alkaloids and Their Mechanism in Neurodegenerative Disorders. Int J Biol Sci. 2018;14(3):341-357 Authors: Hussain G, Rasul A, Anwar H, Aziz N, Razzaq A, Wei W, Ali M, Li J, Li X Abstract Neurodegenerative diseases are conventionally demarcated as disorders with selective loss of ... read more

Related ArticlesCommentary on Giralt et al.: PTK2B/Pyk2 overexpression improves a mouse model of Alzheimer's disease. Exp Neurol. 2019 01;311:313-317 Authors: Polis B, Gil-Henn H Abstract Alzheimer's disease (AD) is a progressive neurodegenerative disorder that is the most common cause of dementia and the 6th leading cause of death. Although ... read more

Related ArticlesHuntington's disease: Managing neuropsychiatric symptoms in Huntington's disease. Australas Psychiatry. 2018 Aug;26(4):376-380 Authors: Loi SM, Walterfang M, Velakoulis D, Looi JC Abstract OBJECTIVES: This clinical update review focuses on the management of the neuropsychiatric manifestations of Huntington's disease (HD). The review highlights current issues regarding pharmacological and non-pharmacological ... read more

Related ArticlesROCK inhibition in models of neurodegeneration and its potential for clinical translation. Pharmacol Ther. 2018 09;189:1-21 Authors: Koch JC, Tatenhorst L, Roser AE, Saal KA, Tönges L, Lingor P Abstract Neurodegenerative disorders like Parkinson's disease, Alzheimer's disease, or amyotrophic lateral sclerosis are affecting a rapidly increasing population ... read more

Circadian Clocks Function in Concert with Heat Shock Organizing Protein to Modulate Mutant Huntingtin Aggregation and Toxicity. Cell Rep. 2019 Apr 02;27(1):59-70.e4 Authors: Xu F, Kula-Eversole E, Iwanaszko M, Hutchison AL, Dinner A, Allada R Abstract Neurodegenerative diseases commonly involve the disruption of circadian rhythms. Studies indicate that mutant Huntingtin ... read more

Related ArticlesPositive allosteric modulation of the type 1 cannabinoid receptor reduces the signs and symptoms of Huntington's disease in the R6/2 mouse model. Neuropharmacology. 2019 Mar 30;: Authors: Laprairie RB, Bagher AM, Rourke JL, Zrein A, Cairns EA, Kelly MEM, Sinal CJ, Kulkarni PM, Thakur GA, Denovan-Wright EM Abstract Huntington's ... read more

Related ArticlesImpaired Redox Signaling in Huntington's Disease: Therapeutic Implications. Front Mol Neurosci. 2019;12:68 Authors: Paul BD, Snyder SH Abstract Huntington's disease (HD) is a neurodegenerative disease triggered by expansion of polyglutamine repeats in the protein huntingtin. Mutant huntingtin (mHtt) aggregates and elicits toxicity by multiple mechanisms which range from dysregulated ... read more

Related ArticlesProgressive microstructural changes of the occipital cortex in Huntington's disease. Brain Imaging Behav. 2018 Dec;12(6):1786-1794 Authors: Odish OFF, Reijntjes RHAM, van den Bogaard SJA, Roos RAC, Leemans A Abstract In this study we longitudinally investigated the rate of microstructural alterations in the occipital cortex in different stages ... read more

Related ArticlesMitochondrial Dysfunction in Huntington's Disease; Interplay Between HSF1, p53 and PGC-1α Transcription Factors. Front Cell Neurosci. 2019;13:103 Authors: Intihar TA, Martinez EA, Gomez-Pastor R Abstract Huntington's disease (HD) is a neurodegenerative disease caused by an expanded CAG repeat in the huntingtin (HTT) gene, causing the protein to misfold and ... read more

Related ArticlesCombination of stem cell and gene therapy ameliorates symptoms in Huntington's disease mice. NPJ Regen Med. 2019;4:7 Authors: Cho IK, Hunter CE, Ye S, Pongos AL, Chan AWS Abstract Huntington's disease (HD) is a dominantly inherited monogenetic disorder characterized by motor and cognitive dysfunction due to neurodegeneration. The disease ... read more

Related ArticlesImpaired Remyelination in a Mouse Model of Huntington Disease. Mol Neurobiol. 2019 Apr 02;: Authors: Teo RTY, Ferrari Bardile C, Tay YL, Yusof NABM, Kreidy CA, Tan LJ, Pouladi MA Abstract White matter (WM) abnormalities are a well-established feature of Huntington disease (HD), although their nature is not fully ... read more

Related ArticlesOxysterol research: a brief review. Biochem Soc Trans. 2019 Apr 01;: Authors: Griffiths WJ, Wang Y Abstract In the present study, we discuss the recent developments in oxysterol research. Exciting results have been reported relating to the involvement of oxysterols in the fields of neurodegenerative disease, especially in Huntington's ... read more

Related ArticlesMetabotropic glutamate receptor 5 ablation accelerates age-related neurodegeneration and neuroinflammation. Neurochem Int. 2019 Mar 28;: Authors: Carvalho TG, Alves-Silva J, Souza JM, Real ALCV, Doria JG, Vieira ELM, Gomes GF, de Oliveira AC, Miranda AS, Ribeiro FM Abstract The growing elderly population world widely prompts the need for studies ... read more

Prevalence of Carriers of Intermediate and Pathological Polyglutamine Disease-Associated Alleles Among Large Population-Based Cohorts. JAMA Neurol. 2019 Apr 01;: Authors: Gardiner SL, Boogaard MW, Trompet S, de Mutsert R, Rosendaal FR, Gussekloo J, Jukema JW, Roos RAC, Aziz NA Abstract Importance: Nine hereditary neurodegenerative diseases are known as polyglutamine diseases, ... read more

Related ArticlesMetabolic Reprogramming in Astrocytes Distinguishes Region-Specific Neuronal Susceptibility in Huntington Mice. Cell Metab. 2019 Mar 26;: Authors: Polyzos AA, Lee DY, Datta R, Hauser M, Budworth H, Holt A, Mihalik S, Goldschmidt P, Frankel K, Trego K, Bennett MJ, Vockley J, Xu K, Gratton E, McMurray CT Abstract The ... read more

Visual Dysfunction in Huntington's Disease: A Systematic Review. J Huntingtons Dis. 2019 Mar 28;: Authors: Dhalla A, Pallikadavath S, V Hutchinson C Abstract It is well-documented that patients with Huntington's disease (HD) exhibit specific deficits in visual cognition. A less well-documented literature also exists that suggests people with HD experience ... read more

Concurrent Cross-Sectional and Longitudinal Analyses of Multivariate White Matter Profiles and Clinical Functioning in Pre-Diagnosis Huntington Disease. J Huntingtons Dis. 2019 Mar 28;: Authors: Ciarochi JA, Johnson HJ, Calhoun VD, Liu J, Espinoza FA, Bockholt HJ, Misiura M, Caprihan A, Plis S, Paulsen JS, A Turner J, PREDICT-HD Investigators and ... read more

Related ArticlesPerspectives of Cannabinoid Type 2 Receptor (CB2R) Ligands in Neurodegenerative Disorders: Structure-Affinity Relationship (SAfiR) and Structure-Activity Relationship (SAR) Studies. J Med Chem. 2017 12 28;60(24):9913-9931 Authors: Spinelli F, Capparelli E, Abate C, Colabufo NA, Contino M Abstract Up-regulation of CB2R on activated microglial cells, the first step in ... read more

Related ArticlesSesamin and sesamol attenuate H2O2 -induced oxidative stress on human neuronal cells via the SIRT1-SIRT3-FOXO3a signaling pathway. Nutr Neurosci. 2019 Mar 30;:1-12 Authors: Ruankham W, Suwanjang W, Wongchitrat P, Prachayasittikul V, Prachayasittikul S, Phopin K Abstract BACKGROUND: An imbalance of free radicals and antioxidant defense systems in physiological processes ... read more

Related ArticlesNeurodegenerative Disorders Treatment: The MicroRNA Role. Curr Gene Ther. 2017;17(5):327-363 Authors: Ridolfi B, Abdel-Haq H Abstract Neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Huntington's disease and prion disease are not timely and effectively treated using conventional therapies. This emphasizes the need for alternative ... read more

Related ArticlesGrey matter volume loss is associated with specific clinical motor signs in Huntington's disease. Parkinsonism Relat Disord. 2018 01;46:56-61 Authors: Coppen EM, Jacobs M, van den Berg-Huysmans AA, van der Grond J, Roos RAC Abstract BACKGROUND: Motor disturbances are clinical hallmarks of Huntington's disease (HD) and involve chorea, ... read more

Using stem cell-derived neurons in drug screening for neurological diseases. Neurobiol Aging. 2019 Feb 20;78:130-141 Authors: Little D, Ketteler R, Gissen P, Devine MJ Abstract Induced pluripotent stem cells and their derivatives have become an important tool for researching disease mechanisms. It is hoped that they could be used to ... read more