RSS Feed – Literature

Obsessive-Compulsive and Perseverative Behaviors in Huntington's Disease. J Huntingtons Dis. 2019 Jan 31;: Authors: Oosterloo M, Craufurd D, Nijsten H, van Duijn E Abstract BACKGROUND: Neuropsychiatric symptoms are highly prevalent in Huntington's disease (HD). However, little is known of the prevalence and course of obsessive-compulsive behaviors (OCBs) and perseverative behaviors ... read more

Related ArticlesIdentification of Altered Developmental Pathways in Human Juvenile HD iPSC With 71Q and 109Q Using Transcriptome Profiling. Front Cell Neurosci. 2018;12:528 Authors: Świtońska K, Szlachcic WJ, Handschuh L, Wojciechowski P, Marczak Ł, Stelmaszczuk M, Figlerowicz M, Figiel M Abstract In Huntington disease (HD) subtle symptoms in patients may occur ... read more

Related ArticlesEukaryotic (translation) initiation factor 4A (eIF4A) inhibition may be used to treat Huntington's disease by decreasing the expression of huntingtin. Med Hypotheses. 2018 05;114:28 Authors: Webb TE PMID: 29602458 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesMutant Exon1 Huntingtin Aggregation is Regulated by T3 Phosphorylation-Induced Structural Changes and Crosstalk between T3 Phosphorylation and Acetylation at K6. Angew Chem Int Ed Engl. 2017 05 02;56(19):5202-5207 Authors: Chiki A, DeGuire SM, Ruggeri FS, Sanfelice D, Ansaloni A, Wang ZM, Cendrowska U, Burai R, Vieweg S, Pastore ... read more

Related ArticlesBrain-Derived Neurotrophic Factor Prevents Depressive-Like Behaviors in Early-Symptomatic YAC128 Huntington's Disease Mice. Mol Neurobiol. 2018 Sep;55(9):7201-7215 Authors: da Fonsêca VS, da Silva Colla AR, de Paula Nascimento-Castro C, Plácido E, Rosa JM, Farina M, Gil-Mohapel J, Rodrigues ALS, Brocardo PS Abstract Huntington disease (HD) is a neurodegenerative disorder ... read more

Related ArticlesHuntington Disease as a Neurodevelopmental Disorder and Early Signs of the Disease in Stem Cells. Mol Neurobiol. 2018 Apr;55(4):3351-3371 Authors: Wiatr K, Szlachcic WJ, Trzeciak M, Figlerowicz M, Figiel M Abstract Huntington disease (HD) is a dominantly inherited disorder caused by a CAG expansion mutation in the ... read more

Related ArticlesHuntingtin protein: A new option for fixing the Huntington's disease countdown clock. Neuropharmacology. 2018 06;135:126-138 Authors: Caterino M, Squillaro T, Montesarchio D, Giordano A, Giancola C, Melone MAB Abstract Huntington's disease is a dreadful, incurable disorder. It springs from the autosomal dominant mutation in the first exon of ... read more

Deregulated Splicing Is a Major Mechanism of RNA-Induced Toxicity in Huntington's Disease. J Mol Biol. 2019 Jan 31;: Authors: Schilling J, Broemer M, Atanassov I, Duernberger Y, Vorberg I, Dieterich C, Dagane A, Dittmar G, Wanker E, van Roon-Mom W, Winter J, Krauß S Abstract Huntington's disease (HD) is caused ... read more

Altered expression of dopaminergic cell fate regulating genes prior to manifestation of symptoms in a transgenic rat model of Huntington's disease. Brain Res. 2019 Jan 31;: Authors: Huguet G, Temel Y, Kádár E, Pol S, Casaca-Carreira J, Segura-Torres P, Jahanshahi A Abstract Hyperactivity of the dopaminergic pathway is thought to ... read more

Related ArticlesAluminum in Neurological and Neurodegenerative Disease. Mol Neurobiol. 2019 Jan 31;: Authors: McLachlan DRC, Bergeron C, Alexandrov PN, Walsh WJ, Pogue AI, Percy ME, Kruck TPA, Fang Z, Sharfman NM, Jaber V, Zhao Y, Li W, Lukiw WJ Abstract With continuing cooperation from 18 domestic and international brain banks ... read more

The Role of PI3K/Akt and ERK in Neurodegenerative Disorders. Neurotox Res. 2019 Feb 01;: Authors: Rai SN, Dilnashin H, Birla H, Singh SS, Zahra W, Rathore AS, Singh BK, Singh SP Abstract Disruption of Akt and Erk-mediated signal transduction significantly contributes in the pathogenesis of various neurodegenerative diseases (NDs), such ... read more

Related ArticlesCross-sectional and longitudinal voxel-based grey matter asymmetries in Huntington's disease. Neuroimage Clin. 2018;17:312-324 Authors: Minkova L, Gregory S, Scahill RI, Abdulkadir A, Kaller CP, Peter J, Long JD, Stout JC, Reilmann R, Roos RA, Durr A, Leavitt BR, Tabrizi SJ, Klöppel S, TRACK-HD Investigators Abstract Huntington's disease ... read more

Related ArticlesDesign, Construction, and Application of Transcription Activation-Like Effectors. Methods Mol Biol. 2019;1937:47-58 Authors: Deng P, Carter S, Fink K Abstract Transcription activator-like effectors (TALEs) are modular proteins derived from the plant Xanthomonas sp. pathogen that can be designed to target unique DNA sequences following a simple cipher. Customized TALE ... read more

Related ArticlesTexture Classification and Visualization of Time Series of Gait Dynamics in Patients With Neuro-Degenerative Diseases. IEEE Trans Neural Syst Rehabil Eng. 2018 01;26(1):188-196 Authors: Pham TD Abstract The analysis of gait dynamics is helpful for predicting and improving the quality of life, morbidity, and mortality in neuro-degenerative patients. ... read more

Related ArticlesApplication of Monoterpenoids and their Derivatives for Treatment of Neurodegenerative Disorders. Curr Med Chem. 2018;25(39):5327-5346 Authors: Volcho KP, Laev SS, Ashraf GM, Aliev G, Salakhutdinov NF Abstract Neurodegenerative disorders (NDDs) like Alzheimer's disease, Parkinson's disease and Huntington's disease are a heterogeneous group of disorders with the progressive ... read more

Related ArticlesPerturbations in the p53/miR-34a/SIRT1 pathway in the R6/2 Huntington's disease model. Mol Cell Neurosci. 2018 04;88:118-129 Authors: Reynolds RH, Petersen MH, Willert CW, Heinrich M, Nymann N, Dall M, Treebak JT, Björkqvist M, Silahtaroglu A, Hasholt L, Nørremølle A Abstract The three factors, p53, the microRNA-34 family and ... read more

Related ArticlesA tetracycline-dependent ribozyme switch allows conditional induction of gene expression in Caenorhabditis elegans. Nat Commun. 2019 Jan 30;10(1):491 Authors: Wurmthaler LA, Sack M, Gense K, Hartig JS, Gamerdinger M Abstract The nematode Caenorhabditis elegans represents an important research model. Convenient methods for conditional induction of gene expression in this ... read more

Related ArticlesComparison of the Toxic Effects of Quinolinic Acid and 3-Nitropropionic Acid in C. elegans: Involvement of the SKN-1 Pathway. Neurotox Res. 2018 02;33(2):259-267 Authors: Kotlar I, Colonnello A, Aguilera-González MF, Avila DS, de Lima ME, García-Contreras R, Ortíz-Plata A, Soares FAA, Aschner M, Santamaría A Abstract The tryptophan ... read more

Related ArticlesAberrant subcellular localization of SQSTM1/p62 contributes to increased vulnerability to proteotoxic stress recovery in Huntington's disease. Mol Cell Neurosci. 2018 04;88:43-52 Authors: Huang N, Erie C, Lu ML, Wei J Abstract Proteotoxic stress plays an important role in the pathogenesis of Huntington's disease (HD). Autophagy is proposed as ... read more

Related ArticlesGenetic testing for Huntington's disease: A thematic analysis of online support community messages. J Health Psychol. 2019 Jan 30;:1359105319826340 Authors: Smedley RM, Coulson NS Abstract Huntington's disease is a fatal late-onset genetic illness that causes motor, cognitive and psychiatric disorders. Individuals considering genetic testing may benefit from online social ... read more

Related ArticlesIs it better to die than to be lonely? J Med Ethics. 2017 09;43(9):575-576 Authors: Roache R PMID: 28851807 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesTime-Restricted Feeding Improves Circadian Dysfunction as well as Motor Symptoms in the Q175 Mouse Model of Huntington's Disease. eNeuro. 2018 Jan-Feb;5(1): Authors: Wang HB, Loh DH, Whittaker DS, Cutler T, Howland D, Colwell CS Abstract Huntington's disease (HD) patients suffer from a progressive neurodegeneration that results in cognitive, ... read more

Neurological profiles beyond the sleep disorder in patients with anti-IgLON5 disease. Curr Opin Neurol. 2019 Jan 28;: Authors: Gaig C, Compta Y Abstract PURPOSE OF REVIEW: Anti-IgLON5 disease is a novel entity characterized by a distinctive sleep disorder associated with a variety of neurological symptoms, antibodies against IgLON5, and pathological ... read more

Attitudes of Potential Participants Towards Molecular Therapy Trials in Huntington's Disease. J Huntingtons Dis. 2019 Jan 21;: Authors: Bardakjian TM, Naczi KF, Gonzalez-Alegre P Abstract BACKGROUND: Advances in molecular therapeutic approaches in the last decade are translating into the design of non-traditional clinical trials. In order to improve their feasibility, ... read more

Mitophagy links oxidative stress conditions and neurodegenerative diseases. Neural Regen Res. 2019 May;14(5):749-756 Authors: Shefa U, Jeong NY, Song IO, Chung HJ, Kim D, Jung J, Huh Y Abstract Mitophagy is activated by a number of stimuli, including hypoxia, energy stress, and increased oxidative phosphorylation activity. Mitophagy is associated with ... read more

Related ArticlesCerebrospinal fluid flow dynamics in Huntington's disease evaluated by phase contrast MRI. Eur J Neurosci. 2019 Jan 28;: Authors: Rodrigues FB, Byrne LM, De Vita E, Johnson EB, Hobbs NZ, Thornton JS, Scahill RI, Wild EJ Abstract Multiple targeted therapeutics for Huntington's disease are now in clinical trials, including ... read more

Activity Behaviour of Minipigs Transgenic for the Huntington Gene. J Huntingtons Dis. 2019 Jan 24;: Authors: Rieke L, Fels M, Schubert R, Habbel B, Matheis T, Schuldenzucker V, Kemper N, Reilmann R Abstract BACKGROUND: To increase the reliability of translating preclinical findings to humans, large genetic animals, such as the ... read more

Induced pluripotent stem cells from Huntington's disease patients: a promising approach to define and correct disease-related alterations. Neural Regen Res. 2019 May;14(5):769-770 Authors: Fatima A, Gutiérrez-Garcia R, Vilchez D AbstractPMID: 30688260 [PubMed] ... read more

Related ArticlesMetabolic signatures of Huntington's disease (HD): (1)H NMR analysis of the polar metabolome in post-mortem human brain. Biochim Biophys Acta. 2016 09;1862(9):1675-84 Authors: Graham SF, Kumar PK, Bjorndahl T, Han B, Yilmaz A, Sherman E, Bahado-Singh RO, Wishart D, Mann D, Green BD Abstract Huntington's disease (HD) ... read more

Risk Assessment for Huntington's Disease for (Future) Offspring Requires Offering Preconceptional CAG Analysis to Both Partners. J Huntingtons Dis. 2019 Nov 24;: Authors: Tibben A, Dondorp WJ, de Wert GM, de Die-Smulders CE, Losekoot M, Bijlsma EK Abstract Amongst the main reasons people at risk for Huntington's disease (HD) have ... read more

Related ArticlesA selective inhibitor of histone deacetylase 3 prevents cognitive deficits and suppresses striatal CAG repeat expansions in Huntington's disease mice. Sci Rep. 2017 07 20;7(1):6082 Authors: Suelves N, Kirkham-McCarthy L, Lahue RS, Ginés S Abstract Huntington's disease (HD) is a neurodegenerative disorder whose major symptoms include progressive ... read more

Related ArticlesPolyglutamine expansion affects huntingtin conformation in multiple Huntington's disease models. Sci Rep. 2017 07 11;7(1):5070 Authors: Daldin M, Fodale V, Cariulo C, Azzollini L, Verani M, Martufi P, Spiezia MC, Deguire SM, Cherubini M, Macdonald D, Weiss A, Bresciani A, Vonsattel JG, Petricca L, Marsh JL, Gines ... read more

Related ArticlesMetabolite mapping reveals severe widespread perturbation of multiple metabolic processes in Huntington's disease human brain. Biochim Biophys Acta. 2016 09;1862(9):1650-62 Authors: Patassini S, Begley P, Xu J, Church SJ, Reid SJ, Kim EH, Curtis MA, Dragunow M, Waldvogel HJ, Snell RG, Unwin RD, Faull RL, Cooper GJ ... read more

Related ArticlesImpact of epigenetics in aging and age related neurodegenerative diseases. Front Biosci (Landmark Ed). 2018 03 01;23:1445-1464 Authors: Gangisetty O, Cabrera MA, Murugan S Abstract Epigenetics involves multiple processes such as DNA methylation, histone code modifications, and noncoding RNAs to regulate gene expression. In recent years the implications ... read more

Related ArticlesExploring emotion regulation and emotion recognition in people with presymptomatic Huntington's disease: The role of emotional awareness. Neuropsychologia. 2018 04;112:1-9 Authors: Zarotti N, Simpson J, Fletcher I, Squitieri F, Migliore S Abstract Interest in the role of both emotion regulation and recognition in our understanding of mental health ... read more

Stem cells in animal models of Huntington disease: A systematic review. Mol Cell Neurosci. 2019 Jan 24;: Authors: Colpo GD, Stimming EF, Teixeira AL Abstract Huntington's disease (HD) is an autosomal-dominant neurodegenerative disorder encoding a mutant form of the huntingtin protein (HTT). HD is pathologically characterized by loss of neurons ... read more

Effect of ozone exposure on dendritic spines of CA1 pyramidal neurons of the dorsal hippocampus and on object-place recognition memory in rats. Neuroscience. 2019 Jan 24;: Authors: Bello-Medina PC, Prado-Alcalá RA, Rivas-Arancibia S Abstract The growth of many cities has generated an increase in the emission of environmental pollutants. Exposure ... read more

Related ArticlesMitochondrial distribution violation and nuclear indentations in neurons differentiated from iPSCs of Huntington's disease patients. J Stem Cells Regen Med. 2018;14(2):80-85 Authors: Nekrasov ED, Kiselev SL Abstract AIM: Huntington's disease (HD) is an inherited disease caused by an expansion of cytosine-adenine-guanine (CAG) repeats in the huntingtin gene (HTT) that ... read more

Related ArticlesEmerging differences between Huntington's disease-like 2 and Huntington's disease: A comparison using MRI brain volumetry. Neuroimage Clin. 2019 Jan 07;21:101666 Authors: Anderson DG, Haagensen M, Ferreira-Correia A, Pierson R, Carr J, Krause A, Margolis RL Abstract Huntington's Disease-Like 2 (HDL2), caused by a CTG/CAG expansion in JPH3 on chromosome ... read more

Related ArticlesOperationalizing compensation over time in neurodegenerative disease. Brain. 2017 04 01;140(4):1158-1165 Authors: Gregory S, Long JD, Klöppel S, Razi A, Scheller E, Minkova L, Papoutsi M, Mills JA, Durr A, Leavitt BR, Roos RAC, Stout JC, Scahill RI, Langbehn DR, Tabrizi SJ, Rees G PMID: 28334888 [PubMed ... read more

Related ArticlesOverexpression of a conserved HSP40 chaperone reduces toxicity of several neurodegenerative disease proteins. Prion. 2018 01 02;12(1):16-22 Authors: Park SK, Arslan F, Kanneganti V, Barmada SJ, Purushothaman P, Verma SC, Liebman SW Abstract TDP-43 and FUS are DNA/RNA binding proteins associated with neuronal inclusions in amyotrophic lateral sclerosis ... read more

Related Articles[Telemonitoring of swallowing function: technologies in speech therapy practice.] Recenti Prog Med. 2018 Feb;109(2):146-148 Authors: Tedesco A, Lavermicocca V, Notarnicola M, De Francesco L, Dellomonaco AR Abstract The process of medical-healthcare technological revolution represents an advantage for the patient and for the care provider, in terms of costs ... read more

Related ArticlesLoss of striatal 90-kDa ribosomal S6 kinase (Rsk) is a key factor for motor, synaptic and transcription dysfunction in Huntington's disease. Biochim Biophys Acta. 2016 07;1862(7):1255-66 Authors: Anglada-Huguet M, Giralt A, Rué L, Alberch J, Xifró X Abstract Huntington's disease (HD) is characterized by motor dysfunction due ... read more

Related ArticlesStructural insights into the aggregation mechanism of huntingtin exon 1 protein fragment with different polyQ-lengths. J Cell Biochem. 2019 Jan 22;: Authors: Priya SB, Gromiha MM Abstract Huntington disease is a neurodegenerative disorder caused by the expansion of polyglutamine (polyQ) at the N-terminal of the huntingtin exon 1 protein. ... read more

Related ArticlesReview: Somatic mutations in neurodegeneration. Neuropathol Appl Neurobiol. 2018 04;44(3):267-285 Authors: Leija-Salazar M, Piette C, Proukakis C Abstract Somatic mutations are postzygotic mutations which may lead to mosaicism, the presence of cells with genetic differences in an organism. Their role in cancer is well established, but detailed investigation ... read more

Related ArticlesKynurenine 3-Monooxygenase Activity in Human Primary Neurons and Effect on Cellular Bioenergetics Identifies New Neurotoxic Mechanisms. Neurotox Res. 2019 Jan 21;: Authors: Castellano-Gonzalez G, Jacobs KR, Don E, Cole NJ, Adams S, Lim CK, Lovejoy DB, Guillemin GJ Abstract Upregulation of the kynurenine pathway (KP) of tryptophan metabolism is ... read more

Related ArticlesManganese Enhanced MRI for Use in Studying Neurodegenerative Diseases. Front Neural Circuits. 2018;12:114 Authors: Saar G, Koretsky AP Abstract MRI has been extensively used in neurodegenerative disorders, such as Alzheimer's disease (AD), frontal-temporal dementia (FTD), mild cognitive impairment (MCI), Parkinson's disease (PD), Huntington's disease (HD) and amyotrophic lateral sclerosis ... read more

Related ArticlesNew insights into the role of microRNAs and long noncoding RNAs in most common neurodegenerative diseases. J Cell Biochem. 2019 Jan 20;: Authors: Maniati MS, Maniati M, Yousefi T, Ahmadi-Ahangar A, Tehrani SS Abstract Neurodegenerative diseases (NDs) are a diversity of neurological disorders characterized by the progressive degeneration of ... read more

Chemical neuroanatomy of the substantia nigra in the ovine brain. J Chem Neuroanat. 2019 Jan 18;: Authors: Murray SJ, Black BL, Reid SJ, Rudiger SR, Bawden CS, Snell RG, Waldvogel HJ, Faull RLM Abstract The substantia nigra is an integral component of the basal ganglia circuitry for limbic and motor ... read more

Related ArticlesHuman Pluripotent Stem Cell-Derived Striatal Interneurons: Differentiation and Maturation In Vitro and in the Rat Brain. Stem Cell Reports. 2019 Jan 02;: Authors: Noakes Z, Keefe F, Tamburini C, Kelly CM, Cruz Santos M, Dunnett SB, Errington AC, Li M Abstract Striatal interneurons are born in the medial and caudal ... read more