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Related ArticlesDirect assessment of presynaptic modulation of cortico-striatal glutamate release in a Huntington's disease mouse model. J Neurophysiol. 2018 Oct 17;: Authors: Koch ET, Woodard CL, Raymond LA Abstract Glutamate is the main excitatory neurotransmitter in the brain, and impairments in its signaling are associated with many neurological disorders, including ... read more

Related ArticlesIntroducing an expanded CAG tract into the huntingtin gene causes a wide spectrum of ultrastructural defects in cultured human cells. PLoS One. 2018;13(10):e0204735 Authors: Morozova KN, Suldina LA, Malankhanova TB, Grigor'eva EV, Zakian SM, Kiseleva E, Malakhova AA Abstract Modeling of neurodegenerative diseases in vitro holds great promise for ... read more

Related ArticlesTrends over 42 years in the Adult Medical Genetics Clinic at the University of Washington. Genet Med. 2018 Oct 16;: Authors: Gay EA, Byers PH, Bennett RL, Bird TD, Hisama FM Abstract PURPOSE: We analyzed the patients served by the University of Washington Adult Genetic Medicine Clinic (UWAGMC) over ... read more

Related ArticlesR-loops: targets for nuclease cleavage and repeat instability. Curr Genet. 2018 Aug;64(4):789-794 Authors: Freudenreich CH Abstract R-loops form when transcribed RNA remains bound to its DNA template to form a stable RNA:DNA hybrid. Stable R-loops form when the RNA is purine-rich, and are further stabilized by DNA secondary ... read more

Selection of Reference Regions to Model Neurodegeneration in Huntington Disease by 18F-FDG PET/CT Using Imaging and Clinical Parameters. Clin Nucl Med. 2018 Oct 15;: Authors: López Mora DA, Sampedro F, Camacho V, Fernández A, Fuentes F, Duch J, Pérez-Perez J, Martínez-Horta S, Marín-Lahoz J, Domènech A, Flotats A, Estorch M, ... read more

Related ArticlesModulation of AMPA receptor surface diffusion restores hippocampal plasticity and memory in Huntington's disease models. Nat Commun. 2018 Oct 15;9(1):4272 Authors: Zhang H, Zhang C, Vincent J, Zala D, Benstaali C, Sainlos M, Grillo-Bosch D, Daburon S, Coussen F, Cho Y, David DJ, Saudou F, Humeau Y, Choquet D ... read more

Related ArticlesNeuroimaging of Sleep Disturbances in Movement Disorders. Front Neurol. 2018;9:767 Authors: Yousaf T, Pagano G, Wilson H, Politis M Abstract Sleep dysfunction is recognized as a distinct clinical manifestation in movement disorders, often reported early on in the disease course. Excessive daytime sleepiness, rapid eye movement sleep behavior disorder ... read more

Related ArticlesASK1 in neurodegeneration. Adv Biol Regul. 2017 Dec;66:63-71 Authors: Guo X, Namekata K, Kimura A, Harada C, Harada T Abstract Neurodegenerative diseases (NDDs) such as glaucoma, multiple sclerosis (MS), Alzheimer's disease (AD), Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), and Huntington's disease (HD) are characterized by the progressive ... read more

Related ArticlesPublisher Correction: Deubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's disease. Nat Commun. 2018 Oct 15;9(1):4333 Authors: Aron R, Pellegrini P, Green EW, Maddison DC, Opoku-Nsiah K, Wong JS, Daub AC, Giorgini F, Finkbeiner S Abstract The original version of this Article incorrectly ... read more

Altered Levels of Long NcRNAs Meg3 and Neat1 in Cell and Animal models of Huntington's Disease. RNA Biol. 2018 Oct 15;: Authors: Chanda K, Das S, Chakraborty J, Bucha S, Maitra A, Chatterjee R, Mukhopadhyay D, Bhattacharyya NP Abstract Altered expression levels of protein-coding genes and microRNAs have been implicated ... read more

Cognitive Performance After a One-Year Multidisciplinary Intensive Rehabilitation Program for Huntington's Disease: An Observational Study. J Huntingtons Dis. 2018 Sep 29;: Authors: van Walsem MR, Piira A, Mikalsen G, Fossmo HL, Howe EI, Knutsen SF, Frich JC Abstract BACKGROUND: Studies of physical therapy and multidisciplinary rehabilitation programs for Huntington's disease ... read more

Selective Neuronal Uptake and Distribution of AAVrh8, AAV9, and AAVrh10 in Sheep After Intra-Striatal Administration. J Huntingtons Dis. 2018 Sep 29;: Authors: Mondo E, Moser R, Gao G, Mueller C, Sena-Esteves M, Sapp E, Pfister E, O'Connell D, Takle K, Erger KE, Liu W, Conlon TJ, DiFiglia M, Gounis MJ, ... read more

Related ArticlesPotential molecular consequences of transgene integration: The R6/2 mouse example. Sci Rep. 2017 01 25;7:41120 Authors: Jacobsen JC, Erdin S, Chiang C, Hanscom C, Handley RR, Barker DD, Stortchevoi A, Blumenthal I, Reid SJ, Snell RG, MacDonald ME, Morton AJ, Ernst C, Gusella JF, Talkowski ME Abstract ... read more

Related ArticlesThe Folding Equilibrium of Huntingtin Exon-1 Monomer Depends on its Polyglutamine Tract. J Biol Chem. 2018 Oct 12;: Authors: Bravo-Arredondo JM, Kegulian NC, Schmidt T, Pandey NK, Situ AJ, Ulmer TS, Langen R Abstract Expansion of the polyglutamine (polyQ) tract in exon 1 of the huntingtin protein (Httex1) leads ... read more

Related ArticlesRepeat length variations in ATXN1 and AR modify disease expression in Alzheimer's disease. Neurobiol Aging. 2018 Sep 15;: Authors: Gardiner SL, Harder AVE, Campman YJM, Trompet S, Gussekloo J, van Belzen MJ, Boogaard MW, Roos RAC, Jansen IE, Pijnenburg YAL, Scheltens P, van der Flier WM, Aziz NA Abstract ... read more

Related ArticlesComplete but not partial inhibition of glutamate transporters exacerbates cortical excitability in the R6/2 mouse model of Huntington's disease. CNS Neurosci Ther. 2018 Oct 11;: Authors: Estrada-Sánchez AM, Castro D, Portillo-Ortiz K, Jang K, Nedjat-Haiem M, Levine MS, Cepeda C Abstract AIM: Deficient glutamate reuptake occurs in the cerebral ... read more

Huntington's disease pig model: Squealing into the spotlight. Mov Disord. 2018 Sep;33(9):1410-1411 Authors: Ratna N, Jain S PMID: 30311975 [PubMed - in process] ... read more

Mutant huntingtin reduction in astrocytes slows disease progression in the bachd conditional huntington's disease mouse model. Hum Mol Genet. 2018 Oct 12;: Authors: Wood TE, Barry J, Yang Z, Cepeda C, Levine MS, Gray M Abstract Neuronal and non-neuronal cells express the huntingtin protein, yet neurodegeneration in Huntington's Disease (HD) ... read more

Related ArticlesQuantification of Total and Mutant Huntingtin Protein Levels in Biospecimens Using a Novel alphaLISA Assay. eNeuro. 2018 Jul-Aug;5(4): Authors: Baldo B, Sajjad MU, Cheong RY, Bigarreau J, Vijayvargia R, McLean C, Perrier AL, Seong IS, Halliday G, Petersén Å, Kirik D Abstract The neurodegenerative Huntington's disease (HD) is caused ... read more

Related ArticlesVMAT2 Inhibitors in Neuropsychiatric Disorders. CNS Drugs. 2018 Oct 10;: Authors: Tarakad A, Jimenez-Shahed J Abstract The basal ganglia and dopaminergic pathways play a central role in hyperkinetic movement disorders. Vesicular monoamine transporter 2 (VMAT2) inhibitors, which deplete dopamine at presynaptic striatal nerve terminals, are a class of drugs ... read more

Related ArticlesEarly enteric neuron dysfunction in mouse and human Huntington disease. Parkinsonism Relat Disord. 2017 01;34:73-74 Authors: Sciacca S, Favellato M, Madonna M, Metro D, Marano M, Squitieri F PMID: 27836713 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesThe Drosophila junctophilin gene is functionally equivalent to its four mammalian counterparts and is a modifier of a Huntingtin poly-Q expansion and the Notch pathway. Dis Model Mech. 2018 01 17;11(1): Authors: Calpena E, López Del Amo V, Chakraborty M, Llamusí B, Artero R, Espinós C, ... read more

Related Articles[Neuropsychiatry Of Movement Disorders]. Rev Colomb Psiquiatr. 2017 Oct;46 Suppl 1:59-68 Authors: Orjuela-Rojas JM, Barrios Vincos GA, Martínez Gallego MA Abstract Movement disorders can be defined as neurological syndromes presenting with excessive or diminished automatic or voluntary movements not related to weakness or spasticity. Both Parkinson's disease (PD) ... read more

Related Articles[Roles of exosomes in Parkinson's disease and amyotrophic lateral sclerosis]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2018 Oct 10;35(5):757-761 Authors: Gu X, Chen Y, Shang H Abstract Exosomes, as a kind of extracellular vesicles generated by inward budding of the endosomes to form multi-vesicular bodies (MVBs), are ... read more

Normalizing glucocorticoid levels attenuates metabolic and neuropathological symptoms in the R6/2 mouse model of huntington's disease. Neurobiol Dis. 2018 Oct 04;: Authors: Dufour BD, McBride JL Abstract Huntington's disease (HD) is a fatal genetic neurological disorder caused by a mutation in the human Huntingtin (HTT) gene. This mutation confers a ... read more

Predictors of simulated driving performance in Huntington's disease. Parkinsonism Relat Disord. 2018 Oct 01;: Authors: Jacobs M, Hart EP, Mejia Miranda Y, Groeneveld GJ, van Gerven JMA, Roos RAC Abstract BACKGROUND: As the disease progresses, patients with Huntington's disease (HD), an inherited neurodegenerative disorder, become less independent in their daily ... read more

Trinucleotide Repeat Expansion Diseases, RNAi, and Cancer. Trends Cancer. 2018 Oct;4(10):684-700 Authors: Murmann AE, Yu J, Opal P, Peter ME Abstract Many neurodegenerative diseases are caused by unstable trinucleotide repeat (TNR) expansions located in disease-associated genes. siRNAs based on CAG repeat expansions effectively kill cancer cell lines in vitro through ... read more

Related ArticlesNovel Imaging Biomarkers for Huntington's Disease and Other Hereditary Choreas. Curr Neurol Neurosci Rep. 2018 Oct 05;18(12):85 Authors: Fazio P, Paucar M, Svenningsson P, Varrone A Abstract PURPOSE OF THE REVIEW: Imaging biomarkers for neurodegenerative disorders are primarily developed with the goal to aid diagnosis, to monitor disease progression, ... read more

Related ArticlesInterplay between α-synuclein amyloid formation and membrane structure. Biochim Biophys Acta Proteins Proteom. 2018 Oct 01;: Authors: O'Leary EI, Lee JC Abstract Amyloid formation is a pathological hallmark of many neurodegenerative diseases, including Alzheimer's, Parkinson's, and Huntington's. While it is unknown how these disorders are initiated, in vitro and ... read more

Epigenetics in neurodegenerative diseases: the role of histone deacetylases. CNS Neurol Disord Drug Targets. 2018 Oct 04;: Authors: Sharma S, Taliyan R, Sarathlal KC Abstract Imbalance in histone acetylation levels and consequently the dysfunction in transcription are associated with a wide variety of neurodegenerative diseases. Histone proteins acetylation and deacetylation ... read more

Related ArticlesA Mitochondria-Associated Oxidative Stress Perspective on Huntington's Disease. Front Mol Neurosci. 2018;11:329 Authors: Zheng J, Winderickx J, Franssens V, Liu B Abstract Huntington's disease (HD) is genetically caused by mutation of the Huntingtin (HTT) gene. At present, the mechanisms underlying the defect of HTT and the development of HD ... read more

Related ArticlesOutcome of cell suspension allografts in a patient with Huntington's disease. Ann Neurol. 2018 Oct 04;: Authors: Maxan A, Mason S, Saint-Pierre M, Smith E, Ho A, Harrower T, Watts C, Tai Y, Pavese N, Savage JC, Tremblay MÈ, Gould P, Rosser AE, Dunnett SB, Piccini P, Barker RA, ... read more

Related ArticlesHuntingtin suppression restores cognitive function in a mouse model of Huntington's disease. Sci Transl Med. 2018 Oct 03;10(461): Authors: Southwell AL, Kordasiewicz HB, Langbehn D, Skotte NH, Parsons MP, Villanueva EB, Caron NS, Østergaard ME, Anderson LM, Xie Y, Cengio LD, Findlay-Black H, Doty CN, Fitsimmons B, Swayze EE, ... read more

Related ArticlesQuinolinic Acid-Induced Huntington Disease-Like Symptoms Mitigated by Potent Free Radical Scavenger Edaravone-a Pilot Study on Neurobehavioral, Biochemical, and Histological Approach in Male Wistar Rats. J Mol Neurosci. 2018 Oct 03;: Authors: Sumathi T, Vedagiri A, Ramachandran S, Purushothaman B Abstract In this study, we demonstrated for the first time ... read more

Related ArticlesTetrabenazine Treatment Patterns and Outcomes for Chorea Associated with Huntington Disease: A Retrospective Chart Review. J Huntingtons Dis. 2018 Sep 24;: Authors: Claassen DO, Iyer RG, Shah-Manek B, DiBonaventura M, Abler V, Sung VW Abstract BACKGROUND: Huntington disease (HD) is a neurodegenerative disorder characterized by motor impairments (including chorea), ... read more

Related ArticlesRemoval of the Mitochondrial Fission Factor Mff Exacerbates Neuronal Loss and Neurological Phenotypes in a Huntington's Disease Mouse Model. PLoS Curr. 2018 Jul 26;10: Authors: Cha MY, Chen H, Chan D Abstract OBJECTIVE: Excessive mitochondrial fission has been associated with several neurodegenerative diseases, including Huntington's disease (HD). Consequently, mitochondrial ... read more

Blood-Brain Barrier: From Physiology to Disease and Back. Physiol Rev. 2019 Jan 01;99(1):21-78 Authors: Sweeney MD, Zhao Z, Montagne A, Nelson AR, Zlokovic BV Abstract The blood-brain barrier (BBB) prevents neurotoxic plasma components, blood cells, and pathogens from entering the brain. At the same time, the BBB regulates transport of ... read more

Related ArticlesComputerised Dynamic Posturography in Premanifest and Manifest individuals with Huntington's Disease. Sci Rep. 2018 Oct 02;8(1):14615 Authors: Reyes A, Salomonczyk D, Teo WP, Medina LD, Bartlett D, Pirogovsky-Turk E, Zaenker P, Bloom JC, Simmons RW, Ziman M, Gilbert PE, Cruickshank T Abstract Evidence from small-scale studies indicates that impairments ... read more

Related ArticlesSUMO-Targeted Ubiquitin Ligases (STUbLs) Reduce the Toxicity and Abnormal Transcriptional Activity Associated With a Mutant, Aggregation-Prone Fragment of Huntingtin. Front Genet. 2018;9:379 Authors: Ohkuni K, Pasupala N, Peek J, Holloway GL, Sclar GD, Levy-Myers R, Baker RE, Basrai MA, Kerscher O Abstract Cell viability and gene expression profiles are ... read more

Related ArticlesUnderstanding the need for assistance with survey completion in people with Huntington disease. Qual Life Res. 2018 03;27(3):801-810 Authors: Hahn EA, Downing NR, Stout JC, Paulsen JS, Ready B, Goodnight S, Lai JS, Miner JA, Carlozzi NE Abstract PURPOSE: In Huntington disease (HD), motor, cognitive, and psychiatric changes ... read more

Related ArticlesEvaluating cognition in individuals with Huntington disease: Neuro-QoL cognitive functioning measures. Qual Life Res. 2018 03;27(3):811-822 Authors: Lai JS, Goodnight S, Downing NR, Ready RE, Paulsen JS, Kratz AL, Stout JC, McCormack MK, Cella D, Ross C, Russell J, Carlozzi NE Abstract PURPOSE: Cognitive functioning impacts health-related quality ... read more

Altered driving performance of symptomatic Huntington's disease gene carriers in simulated road conditions. Traffic Inj Prev. 2018 Oct 01;:1-7 Authors: Jacobs M, Hart EP, Miranda YM, Groeneveld GJ, van Gerven JMA, Roos RAC Abstract OBJECTIVE: In clinical practice, patients with Huntington's disease (HD) often decide to solely drive in their ... read more

Related ArticlesRetinal dysfunction in a presymptomatic patient with Huntington's disease. Doc Ophthalmol. 2018 06;136(3):213-221 Authors: Knapp J, VanNasdale DA, Ramsey K, Racine J Abstract PURPOSE: Huntington's disease (HD) is an autosomal dominant, neurodegenerative disorder characterized by progressive motor dysfunction, cognitive decline, and psychiatric disturbances. Studies have shown retinal abnormalities ... read more

Theranostic applications of nanoparticles in neurodegenerative disorders. Int J Nanomedicine. 2018;13:5561-5576 Authors: Ramanathan S, Archunan G, Sivakumar M, Tamil Selvan S, Fred AL, Kumar S, Gulyás B, Padmanabhan P Abstract The preeminent treatments for neurodegenerative disease are often unavailable due to the poor accessibility of therapeutic drugs. Moreover, the blood-brain ... read more

Related ArticlesRepair kinetics of DNA double-strand breaks and incidence of apoptosis in mouse neural stem/progenitor cells and their differentiated neurons exposed to ionizing radiation. J Radiat Res. 2018 May 01;59(3):261-271 Authors: Kashiwagi H, Shiraishi K, Sakaguchi K, Nakahama T, Kodama S Abstract Neuronal loss leads to neurodegenerative disorders, ... read more

Related ArticlesDeubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's disease. Nat Commun. 2018 Sep 28;9(1):3191 Authors: Aron R, Pellegrini P, Green EW, Maddison DC, Opoku-Nsiah K, Wong JS, Daub AC, Giorgini F, Finkbeiner S Abstract Huntington's disease is a progressive neurodegenerative disorder caused by ... read more

Passive immunotherapies for central nervous system disorders - current delivery challenges and new approaches. Bioconjug Chem. 2018 Sep 28;: Authors: Kumar NN, Pizzo M, Nehra G, Wilken-Resman B, Boroumand S, Thorne R Abstract Passive immunotherapy, i.e., the administration of exogenous antibodies that recognize a specific target antigen, has gained significant ... read more

Related ArticlesCorticopallidal Connectome of the Globus Pallidus Externus in Humans: An Exploratory Study of Structural Connectivity Using Probabilistic Diffusion Tractography. AJNR Am J Neuroradiol. 2018 Sep 27;: Authors: Grewal SS, Holanda VM, Middlebrooks EH Abstract BACKGROUND AND PURPOSE: Electrophysiologic abnormalities of the globus pallidus externus have been shown in several ... read more

Related ArticlesRegulatory mechanisms of incomplete huntingtin mRNA splicing. Nat Commun. 2018 Sep 27;9(1):3955 Authors: Neueder A, Dumas AA, Benjamin AC, Bates GP Abstract Huntington's disease is caused by a CAG repeat expansion in exon 1 of the HTT gene. We have previously shown that exon 1 HTT does not always ... read more

Cilostazol disrupts TLR-4, Akt/GSK-3β/CREB, and IL-6/JAK-2/STAT-3/SOCS-3 crosstalk in a rat model of Huntington's disease. PLoS One. 2018;13(9):e0203837 Authors: El-Abhar H, Abd El Fattah MA, Wadie W, El-Tanbouly DM Abstract Countless neurodegenerative diseases are associated with perverse multiple targets of cyclic nucleotide signalling, hastening neuronal death. Cilostazol, a phosphodiesterase-III inhibitor, exerts ... read more