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Related ArticlesUnderstanding patient-reported outcome measures in Huntington disease: at what point is cognitive impairment related to poor measurement reliability? Qual Life Res. 2018 Jun 16;: Authors: Carlozzi NE, Schilling S, Kratz AL, Paulsen JS, Frank S, Stout JC Abstract PURPOSE: Symptom progression in Huntington disease (HD) is associated with cognitive ... read more

Related ArticlesMechanism of neuroprotection by trehalose: controversy surrounding autophagy induction. Cell Death Dis. 2018 Jun 15;9(7):712 Authors: Lee HJ, Yoon YS, Lee SJ Abstract Trehalose is a non-reducing disaccharide with two glucose molecules linked through an α, α-1,1-glucosidic bond. Trehalose has received attention for the past few decades for its ... read more

Related ArticlesThe Neuropsychology of Huntington's Disease. Arch Clin Neuropsychol. 2017 Nov 01;32(7):876-887 Authors: Snowden JS Abstract Huntington's disease is an inherited, degenerative brain disease, characterized by involuntary movements, cognitive disorder and neuropsychiatric change. Men and women are affected equally. Symptoms emerge at around 40 years, although there is wide ... read more

Related ArticlesUnconventional Secretion and Intercellular Transfer of Mutant Huntingtin. Cells. 2018 Jun 14;7(6): Authors: Tang BL Abstract The mechanism of intercellular transmission of pathological agents in neurodegenerative diseases has received much recent attention. Huntington's disease (HD) is caused by a monogenic mutation in the gene encoding Huntingtin (HTT). Mutant HTT ... read more

Related ArticlesIntravenous immunoglobulin ameliorates motor and cognitive deficits and neuropathology in R6/2 mouse model of Huntington's disease by decreasing mutant huntingtin protein level and normalizing NF-κB signaling pathway. Brain Res. 2018 Jun 11;: Authors: Liu SY, Yu XL, Zhu J, Liu XM, Zhang Y, Dong QX, Ma S, Liu RT ... read more

Related ArticlesTherapeutic strategies for targeting neurodegenerative protein misfolding disorders. Curr Opin Chem Biol. 2018 Jun 11;44:66-74 Authors: Scannevin RH Abstract Neurodegenerative diseases can arise from a multitude of different pathological drivers, however protein misfolding appears to be a common molecular feature central to several disorders. Protein folding, and attainment of ... read more

Related ArticlesRethinking Functional Outcome Measures: The Development of a Novel Upper Limb Token Transfer Test to Assess Basal Ganglia Dysfunction. Front Neurosci. 2018;12:366 Authors: Clinch SP, Busse M, Lelos MJ, Rosser AE Abstract The basal ganglia are implicated in a wide range of motor, cognitive and behavioral activities required for ... read more

Related ArticlesApelin‑13 attenuates ER stress‑associated apoptosis induced by MPP+ in SH‑SY5Y cells. Int J Mol Med. 2018 Jun 05;: Authors: Jiang Y, Liu H, Ji B, Wang Z, Wang C, Yang C, Pan Y, Chen J, Cheng B, Bai B Abstract Apelin‑13, a neuropeptide that acts as a ligand for ... read more

Related ArticlesEpigenetic alterations mediate iPSC normalization of DNA-repair expression and TNR stability in Huntington's disease. J Cell Sci. 2018 Jun 13;: Authors: Mollica PA, Zamponi M, Reid JA, Sharma DK, White AE, Ogle RC, Bruno RD, Sachs PC Abstract Huntington's disease (HD) is a rare autosomal dominant neurodegenerative disorder caused ... read more

Related ArticlesAnaesthesia and orphan disease: airway and anaesthetic management in Huntington's disease. BMJ Case Rep. 2017 Oct 19;2017: Authors: Nguyen PT, Meeks D, Liotiri D Abstract We present a case that highlights the issues surrounding the delivery of a safe general anaesthetic to a patient with Huntington's disease (HD) ... read more

Identification of common coexpression modules based on quantitative network comparison. BMC Bioinformatics. 2018 Jun 13;19(Suppl 8):213 Authors: Jo Y, Kim S, Lee D Abstract BACKGROUND: Finding common molecular interactions from different samples is essential work to understanding diseases and other biological processes. Coexpression networks and their modules directly reflect sample-specific ... read more

Role of non-coding RNAs in non-aging-related neurological disorders. Braz J Med Biol Res. 2018 Jun 11;51(8):e7566 Authors: Vieira AS, Dogini DB, Lopes-Cendes I Abstract Protein coding sequences represent only 2% of the human genome. Recent advances have demonstrated that a significant portion of the genome is actively transcribed as non-coding ... read more

Related ArticlesThe neuroprotective transcription factor ATF5 is decreased and sequestered into polyglutamine inclusions in Huntington's disease. Acta Neuropathol. 2017 Dec;134(6):839-850 Authors: Hernández IH, Torres-Peraza J, Santos-Galindo M, Ramos-Morón E, Fernández-Fernández MR, Pérez-Álvarez MJ, Miranda-Vizuete A, Lucas JJ Abstract Activating transcription factor-5 (ATF5) is a stress-response transcription factor induced upon ... read more

Related ArticlesDual Therapy with Liraglutide and Ghrelin Promotes Brain and Peripheral Energy Metabolism in the R6/2 Mouse Model of Huntington's Disease. Sci Rep. 2018 Jun 12;8(1):8961 Authors: Duarte AI, Sjögren M, Santos MS, Oliveira CR, Moreira PI, Björkqvist M Abstract Neuronal loss alongside altered energy metabolism, are key features of ... read more

Related ArticlesSIRT3 and mitochondrial metabolism in neurodegenerative diseases. Neurochem Int. 2017 Oct;109:184-192 Authors: Salvatori I, Valle C, Ferri A, Carrì MT Abstract The NAD+-dependent deacetylase protein Sirtuin 3 (SIRT3) is emerging among the factors playing a key role in the regulation of mitochondrial function and in the prevention of ... read more

Related ArticlesOxidative metabolism and Ca2+ handling in striatal mitochondria from YAC128 mice, a model of Huntington's disease. Neurochem Int. 2017 Oct;109:24-33 Authors: Hamilton J, Brustovetsky T, Brustovetsky N Abstract The mechanisms implicated in the pathology of Huntington's disease (HD) remain not completely understood, although dysfunction of mitochondrial oxidative metabolism ... read more

Beta catenin is regulated by its subcellular distribution and mutant huntingtin status in Huntington's disease cell STHdhQ111/HdhQ111. Biochem Biophys Res Commun. 2018 Jun 09;: Authors: Ghatak S, Raha S Abstract Dysregulation of gene expression at RNA and protein level is a hallmark of Huntington's disease (HD). Altered levels of microRNAs ... read more

Related ArticlesDisease-modifying effects of ganglioside GM1 in Huntington's disease models. EMBO Mol Med. 2017 Nov;9(11):1537-1557 Authors: Alpaugh M, Galleguillos D, Forero J, Morales LC, Lackey SW, Kar P, Di Pardo A, Holt A, Kerr BJ, Todd KG, Baker GB, Fouad K, Sipione S Abstract Huntington's disease (HD) is ... read more

Related ArticlesThe role of Twist1 in mutant huntingtin-induced transcriptional alterations and neurotoxicity. J Biol Chem. 2018 Jun 11;: Authors: Pan Y, Zhu Y, Yang W, Tycksen E, Liu S, Palucki J, Zhu L, Sasaki Y, Sharma MK, Kim AH, Zhang B, Yano H Abstract Huntington's disease (HD) is a fatal ... read more

Related ArticlesModulation of nuclear REST by alternative splicing: a potential therapeutic target for Huntington's disease. J Cell Mol Med. 2017 Nov;21(11):2974-2984 Authors: Chen GL, Ma Q, Goswami D, Shang J, Miller GM Abstract Huntington's disease (HD) is caused by a genetically mutated huntingtin (mHtt) protein with expanded polyQ ... read more

Related ArticlesDetection of long repeat expansions from PCR-free whole-genome sequence data. Genome Res. 2017 Nov;27(11):1895-1903 Authors: Dolzhenko E, van Vugt JJFA, Shaw RJ, Bekritsky MA, van Blitterswijk M, Narzisi G, Ajay SS, Rajan V, Lajoie BR, Johnson NH, Kingsbury Z, Humphray SJ, Schellevis RD, Brands WJ, Baker ... read more

Cancer: From Wild-Type to Mutant Huntingtin. J Huntingtons Dis. 2018 Jun 04;: Authors: Thion MS, Humbert S Abstract Huntingtin (HTT) is a scaffold protein mostly known because it gives rise to the severe and incurable inherited neurological disorder Huntington's disease (HD) when mutated. The Huntingtin gene (HTT) carries a polymorphic ... read more

Oxidized DJ-1 Levels in Urine Samples as a Putative Biomarker for Parkinson's Disease. Parkinsons Dis. 2018;2018:1241757 Authors: Jang J, Jeong S, Lee SI, Seol W, Seo H, Son I, Ho DH Abstract Parkinson's disease (PD) is the second most common neurodegenerative disease. Oxidative stress is the most critical risk factor ... read more

The Place of PET to Assess New Therapeutic Effectiveness in Neurodegenerative Diseases. Contrast Media Mol Imaging. 2018;2018:7043578 Authors: Dupont AC, Largeau B, Guilloteau D, Santiago Ribeiro MJ, Arlicot N Abstract In vivo exploration of neurodegenerative diseases by positron emission tomography (PET) imaging has matured over the last 20 years, using ... read more

Anorectal Dysfunction in Presymptomatic Mutation Carriers and Patients with Huntington's Disease. J Huntingtons Dis. 2018 Jun 04;: Authors: Kobal J, Matej K, Koželj M, Podnar S Abstract BACKGROUND: Huntington's disease (HD) patients often report anorectal dysfunction; however, in HD research no detailed analysis of these complaints has been published. OBJECTIVE: ... read more

Related ArticlesTrace Oxygen Sensitive Material Based on Two Porphyrin Derivatives in a Heterodimeric Complex. Molecules. 2017 Oct 21;22(10): Authors: Fagadar-Cosma E, Badea V, Fagadar-Cosma G, Palade A, Lascu A, Fringu I, Birdeanu M Abstract The successful preparation of a novel dimer complex formed between 5,10,15,20-tetrakis(3,4-dimethoxyphenyl)-porphyrin Fe(III) chloride and (5,10,15,20-tetraphenylporphinato) dichlorophosphorus(V) ... read more

Related ArticlesProspective Evaluation of Predictive DNA Testing for Huntington's Disease in a Large German Center. J Genet Couns. 2017 Oct;26(5):1029-1040 Authors: Ibisler A, Ocklenburg S, Stemmler S, Arning L, Epplen JT, Saft C, Hoffjan S Abstract We present a prospective study of counselees seeking predictive testing for Huntington's disease ... read more

Related ArticlesFunctional MRI signal fluctuations highlight altered resting brain activity in Huntington's disease. Brain Imaging Behav. 2017 Oct;11(5):1459-1469 Authors: Sarappa C, Salvatore E, Filla A, Cocozza S, Russo CV, Saccà F, Brunetti A, De Michele G, Quarantelli M Abstract The fractional Amplitude of Low Frequency Fluctuations (fALFF) and the ... read more

Related ArticlesSurface-based morphometry reveals caudate subnuclear structural damage in patients with premotor Huntington disease. Brain Imaging Behav. 2017 Oct;11(5):1365-1372 Authors: Kim H, Kim JH, Possin KL, Winer J, Geschwind MD, Xu D, Hess CP Abstract In patients with premotor Huntington's disease (pmHD), literature has reported decreases in caudate ... read more

Related ArticlesEstimation of genetic risk function with covariates in the presence of missing genotypes. Stat Med. 2017 Sep 30;36(22):3533-3546 Authors: Lee AJ, Marder K, Alcalay RN, Mejia-Santana H, Orr-Urtreger A, Giladi N, Bressman S, Wang Y Abstract In genetic epidemiological studies, family history data are collected on relatives of study ... read more

Epigenetic Regulation in Neurodegenerative Diseases. Trends Neurosci. 2018 Jun 06;: Authors: Berson A, Nativio R, Berger SL, Bonini NM Abstract Mechanisms of epigenetic regulation, including DNA methylation, chromatin remodeling, and histone post-translational modifications, are involved in multiple aspects of neuronal function and development. Recent discoveries have shed light on critical ... read more

Related ArticlesSex-dependent behavioral impairments in the HdhQ350/+ mouse line. Behav Brain Res. 2018 Jan 30;337:34-45 Authors: Cao JK, Detloff PJ, Gardner RG, Stella N Abstract Huntington's Disease (HD) is an autosomal dominant neurodegenerative disease characterized by gradual deterioration of motor and cognitive functions and development of psychiatric deficits. Animal ... read more

Sexual behavior and testis morphology in the BACHD rat model. PLoS One. 2018;13(6):e0198338 Authors: Novati A, Yu-Taeger L, Gonzalez Menendez I, Quintanilla Martinez L, Nguyen HP Abstract BACKGROUND: Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a mutation in the huntingtin (HTT) gene, which results in brain ... read more

Related ArticlesThe heat shock response in neurons and astroglia and its role in neurodegenerative diseases. Mol Neurodegener. 2017 09 18;12(1):65 Authors: San Gil R, Ooi L, Yerbury JJ, Ecroyd H Abstract Protein inclusions are a predominant molecular pathology found in numerous neurodegenerative diseases, including amyotrophic lateral sclerosis and ... read more

Related ArticlesResting-state connectivity in neurodegenerative disorders: Is there potential for an imaging biomarker? Neuroimage Clin. 2018;18:849-870 Authors: Hohenfeld C, Werner CJ, Reetz K Abstract Biomarkers in whichever modality are tremendously important in diagnosing of disease, tracking disease progression and clinical trials. This applies in particular for disorders with a long ... read more

Related ArticlesReduced amygdala volumes are related to motor and cognitive signs in Huntington's disease: The IMAGE-HD study. Neuroimage Clin. 2018;18:881-887 Authors: Ahveninen LM, Stout JC, Georgiou-Karistianis N, Lorenzetti V, Glikmann-Johnston Y Abstract In Huntington's disease (HD), the presence of neurodegeneration in brain regions other than the striatum has been recently ... read more

Related ArticlesNeuro-Cardio Mechanisms in Huntington's Disease and Other Neurodegenerative Disorders. Front Physiol. 2018;9:559 Authors: Critchley BJ, Isalan M, Mielcarek M Abstract Although Huntington's disease is generally considered to be a neurological disorder, there is mounting evidence that heart malfunction plays an important role in disease progression. This is perhaps not ... read more

Related ArticlesIntracerebroventricular Administration of L-arginine Improves Spatial Memory Acquisition in Triple Transgenic Mice Via Reduction of Oxidative Stress and Apoptosis. Transl Neurosci. 2018;9:43-53 Authors: Fonar G, Polis B, Meirson T, Maltsev A, Elliott E, Samson AO Abstract Arginine is one of the most versatile semi-essential amino acids. Further to the ... read more

Related ArticlesMitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease. Front Neurosci. 2018;12:342 Authors: Franco-Iborra S, Vila M, Perier C Abstract In recent years, several important advances have been made in our understanding of the pathways that lead to cell dysfunction and death in Parkinson's disease ... read more

Related ArticlesA survey-based study identifies common but unrecognized symptoms in a large series of juvenile Huntington's disease. Neurodegener Dis Manag. 2017 Oct;7(5):307-315 Authors: Moser AD, Epping E, Espe-Pfeifer P, Martin E, Zhorne L, Mathews K, Nance M, Hudgell D, Quarrell O, Nopoulos P Abstract AIM: The symptoms of Huntington's ... read more

Related ArticlesPharmacological activities, mechanisms of action, and safety of salidroside in the central nervous system. Drug Des Devel Ther. 2018;12:1479-1489 Authors: Zhong Z, Han J, Zhang J, Xiao Q, Hu J, Chen L Abstract The primary objective of this review article was to summarize comprehensive information related to the neuropharmacological ... read more

Related ArticlesMeasuring Dynamic Behavior of Trinucleotide Repeat Tracts In Vivo in Saccharomyces cerevisiae. Methods Mol Biol. 2018;1672:439-470 Authors: Williams GM, Surtees JA Abstract Trinucleotide repeat (TNR) tracts are inherently unstable during DNA replication, leading to repeat expansions and/or contractions. Expanded tracts are the cause of over 40 neurodegenerative and ... read more

Related ArticlesBuccal Respiratory Chain Complexes I and IV Quantities in Huntington's Disease Patients. Folia Biol (Praha). 2018;64(1):31-34 Authors: Dušek P, Rodinová M, Lišková I, Klempíř J, Zeman J, Roth J, Hansíková H Abstract Alterations in mitochondrial parameters are an important hallmark of Huntington's disease (HD). The ubiquitous expression of mutant ... read more

Related ArticlesFoxa1 is essential for development and functional integrity of the subthalamic nucleus. Sci Rep. 2016 12 09;6:38611 Authors: Gasser E, Johannssen HC, Rülicke T, Zeilhofer HU, Stoffel M Abstract Inactivation of transcription factor Foxa1 in mice results in neonatal mortality of unknown cause. Here, we report that ... read more

Related ArticlesThe Alteration of Emotion Regulation Precedes the Deficits in Interval Timing in the BACHD Rat Model for Huntington Disease. Front Integr Neurosci. 2018;12:14 Authors: Garces D, El Massioui N, Lamirault C, Riess O, Nguyen HP, Brown BL, Doyère V Abstract Huntington disease (HD) is an autosomal dominantly inherited, progressive ... read more

Gradual Phenotype Development in Huntington Disease Transgenic Minipig Model at 24 Months of Age. Neurodegener Dis. 2018 Jun 05;18(2-3):107-119 Authors: Vidinská D, Vochozková P, Šmatlíková P, Ardan T, Klíma J, Juhás Š, Juhásová J, Bohuslavová B, Baxa M, Valeková I, Motlík J, Ellederová Z Abstract BACKGROUND: Huntington disease (HD) is ... read more

Related ArticlesVergence and Strabismus in Neurodegenerative Disorders. Front Neurol. 2018;9:299 Authors: Kang SL, Shaikh AG, Ghasia FF Abstract Maintaining proper eye alignment is necessary to generate a cohesive visual image. This involves the coordination of complex neural networks, which can become impaired by various neurodegenerative diseases. When the vergence system ... read more

Related ArticlesCannabidiol as a Promising Strategy to Treat and Prevent Movement Disorders? Front Pharmacol. 2018;9:482 Authors: Peres FF, Lima AC, Hallak JEC, Crippa JA, Silva RH, Abílio VC Abstract Movement disorders such as Parkinson's disease and dyskinesia are highly debilitating conditions linked to oxidative stress and neurodegeneration. When available, the ... read more

Related ArticlesThe Self-Inactivating KamiCas9 System for the Editing of CNS Disease Genes. Cell Rep. 2017 Sep 19;20(12):2980-2991 Authors: Merienne N, Vachey G, de Longprez L, Meunier C, Zimmer V, Perriard G, Canales M, Mathias A, Herrgott L, Beltraminelli T, Maulet A, Dequesne T, Pythoud C, Rey M, Pellerin L, ... read more

Related ArticlesA Fully-Automated Subcortical and Ventricular Shape Generation Pipeline Preserving Smoothness and Anatomical Topology. Front Neurosci. 2018;12:321 Authors: Tang X, Luo Y, Chen Z, Huang N, Johnson HJ, Paulsen JS, Miller MI Abstract In this paper, we present a fully-automated subcortical and ventricular shape generation pipeline that acts on structural ... read more