RSS Feed – Literature

Creating Cell and Animal Models of Human Disease by Genome Editing Using CRISPR/Cas9. J Gene Med. 2019 Feb 20;:e3082 Authors: Zarei A, Razban V, Hosseini SE, Tabei SMB Abstract AIMS: Set of unique sequences in bacterial genomes, responsible for protecting bacteria against bacteriophages, have been recently used for genetic manipulation ... read more

Related ArticlesNeuroprotective effect of solanesol against 3-nitropropionic acid-induced Huntington's disease-like behavioral, biochemical, and cellular alterations: Restoration of coenzyme-Q10-mediated mitochondrial dysfunction. Indian J Pharmacol. 2018 Nov-Dec;50(6):309-319 Authors: Mehan S, Monga V, Rani M, Dudi R, Ghimire K Abstract OBJECTIVE: The aim of the present study was to evaluate the solanesol (SNL)-mediated ... read more

Related ArticlesExercise in Huntington's Disease: Current State and Clinical Significance. Tremor Other Hyperkinet Mov (N Y). 2019;9:601 Authors: Mueller SM, Petersen JA, Jung HH Abstract Background: Huntington's disease (HD) is a rare, progressive neurodegenerative disease. Currently, there is no cure for the disease, but treatment may alleviate HD symptoms. In ... read more

Related ArticlesExpanded neurochemical profile in the early stage of Huntington disease using proton magnetic resonance spectroscopy. NMR Biomed. 2018 03;31(3): Authors: Adanyeguh IM, Monin ML, Rinaldi D, Freeman L, Durr A, Lehéricy S, Henry PG, Mochel F Abstract The striatum is a well-known region affected in Huntington disease (HD). ... read more

Huntington's Disease Clinical Trials Corner: January 2019. J Huntingtons Dis. 2019;8(1):115-125 Authors: Rodrigues FB, Quinn L, Wild EJ Abstract In this edition of the Huntington's Disease Clinical Trials Corner we expand on the GENERATION-HD1 and PACE-HD trials, and we list all currently registered and ongoing clinical trials in Huntington's disease.PMID: ... read more

Ghrelin-mediated improvements in the metabolic phenotype in the R6/2 mouse model of Huntington's disease. J Neuroendocrinol. 2019 Feb 18;:e12699 Authors: Rudenko O, Springer C, Skov LJ, Madsen AN, Hasholt L, Nørremølle A, Holst B Abstract Huntington's disease (HD) is a heritable neurodegenerative disorder, characterized by metabolic disturbances, along with cognitive and ... read more

Cognitive Fitness to Drive in Huntington's Disease: Assessing the Clinical Utility of DriveSafe DriveAware. J Huntingtons Dis. 2019;8(1):87-95 Authors: Farrell HJ, Andrews SC, Ryan NP, Davis MC, Gordon S, Stout JC, Fisher F Abstract BACKGROUND: DriveSafe DriveAware (DSDA) has been validated as an off-road screening tool for predicting on-road driving ... read more

Upcoming Meetings Related to Huntington's Disease. J Huntingtons Dis. 2019;8(1):127 Authors: PMID: 30776018 [PubMed - in process] ... read more

Contemporary Dance Practice Improves Motor Function and Body Representation in Huntington's Disease: A Pilot Study. J Huntingtons Dis. 2019;8(1):97-110 Authors: Trinkler I, Chéhère P, Salgues J, Monin ML, Tezenas du Montcel S, Khani S, Gargiulo M, Durr A Abstract BACKGROUND: Physical exercise improves neurological conditions, but adherence is hard to ... read more

Detection of axonal degeneration in a mouse model of Huntington's disease: comparison between diffusion tensor imaging and anomalous diffusion metrics. MAGMA. 2019 Feb 15;: Authors: Gatto RG, Ye AQ, Colon-Perez L, Mareci TH, Lysakowski A, Price SD, Brady ST, Karaman M, Morfini G, Magin RL Abstract OBJECTIVE: The goal of ... read more

Bioenergetic deficits in Huntington's disease iPSC-derived neural cells and rescue with glycolytic metabolites. Hum Mol Genet. 2019 Feb 15;: Authors: Kedaigle AJ, Fraenkel E, Atwal RS, Wu M, Gusella JF, MacDonald ME, Kaye JA, Finkbeiner S, Mattis VB, Tom CM, Svendsen C, King AR, Chen Y, Stocksdale JT, Lim RG, ... read more

Huntingtin associates with the actin cytoskeleton and α-actinin isoforms to influence stimulus dependent morphology changes. PLoS One. 2019;14(2):e0212337 Authors: Tousley A, Iuliano M, Weisman E, Sapp E, Richardson H, Vodicka P, Alexander J, Aronin N, DiFiglia M, Kegel-Gleason KB Abstract One response of cells to growth factor stimulus involves changes ... read more

Apathy Associated With Impaired Recognition of Happy Facial Expressions in Huntington's Disease. J Int Neuropsychol Soc. 2019 Feb 15;:1-9 Authors: Osborne-Crowley K, Andrews SC, Labuschagne I, Nair A, Scahill R, Craufurd D, Tabrizi SJ, Stout JC, TRACK-HD Investigators Abstract OBJECTIVES: Previous research has demonstrated an association between emotion recognition and ... read more

Related ArticlesiNKT Cell Activation Exacerbates the Development of Huntington's Disease in R6/2 Transgenic Mice. Mediators Inflamm. 2019;2019:3540974 Authors: Park HJ, Lee SW, Im W, Kim M, Van Kaer L, Hong S Abstract Huntington's disease (HD) is an inherited neurodegenerative disorder which is caused by a mutation of the huntingtin (HTT) ... read more

Characteristics and advantages of adeno-associated virus vector-mediated gene therapy for neurodegenerative diseases. Neural Regen Res. 2019 Jun;14(6):931-938 Authors: Qu Y, Liu Y, Noor AF, Tran J, Li R Abstract Common neurodegenerative diseases of the central nervous system are characterized by progressive damage to the function of neurons, even leading to ... read more

Related ArticlesA genetic modifier suggests that endurance exercise exacerbates Huntington's disease. Hum Mol Genet. 2018 05 15;27(10):1723-1731 Authors: Corrochano S, Blanco G, Williams D, Wettstein J, Simon M, Kumar S, Moir L, Agnew T, Stewart M, Landman A, Kotiadis VN, Duchen MR, Wackerhage H, Rubinsztein DC, Brown SDM, ... read more

Related ArticlesHesperidin as a Neuroprotective Agent: A Review of Animal and Clinical Evidence. Molecules. 2019 Feb 12;24(3): Authors: Hajialyani M, Hosein Farzaei M, Echeverría J, Nabavi SM, Uriarte E, Sobarzo-Sánchez E Abstract Neuroprotection is the preservation of function and networks of neural tissues from damages caused by various agents, as ... read more

Related ArticlesDJ-1 modulates the unfolded protein response and cell death via upregulation of ATF4 following ER stress. Cell Death Dis. 2019 Feb 12;10(2):135 Authors: Yang J, Kim KS, Iyirhiaro GO, Marcogliese PC, Callaghan SM, Qu D, Kim WJ, Slack RS, Park DS Abstract The unfolded protein response (UPR) triggered by ... read more

Related ArticlesPridopidine Induces Functional Neurorestoration Via the Sigma-1 Receptor in a Mouse Model of Parkinson's Disease. Neurotherapeutics. 2019 Feb 12;: Authors: Francardo V, Geva M, Bez F, Denis Q, Steiner L, Hayden MR, Cenci MA Abstract Pridopidine is a small molecule in clinical development for the treatment of Huntington's disease. ... read more

Motivational Impairment is Accompanied by Corticoaccumbal Dysfunction in the BACHD-Tg5 Rat Model of Huntington's Disease. Cereb Cortex. 2019 Feb 07;: Authors: Zlebnik NE, Gildish I, Sesia T, Fitoussi A, Cole EA, Carson BP, Cachope R, Cheer JF Abstract Neuropsychiatric symptoms, such as avolition, apathy, and anhedonia, precede the onset of ... read more

Related ArticlesFDG-PET in a Case of Very Late-onset Huntington's Disease. Mov Disord Clin Pract. 2018 Mar-Apr;5(2):227-228 Authors: Feil K, Brendel M, Hensler M, Bartenstein P, Seelos K, Dieterich M, Rominger A, Danek A PMID: 30746408 [PubMed] ... read more

Related ArticlesThe "Stutter-Step": A Peculiar Gait Feature in Advanced Huntington's Disease and Chorea-Acanthocytosis. Mov Disord Clin Pract. 2018 Mar-Apr;5(2):223-224 Authors: Termsarasab P, Frucht SJ PMID: 30746406 [PubMed] ... read more

Application of quercetin in neurological disorders: from nutrition to nanomedicine. Rev Neurosci. 2019 Feb 12;: Authors: Amanzadeh E, Esmaeili A, Rahgozar S, Nourbakhshnia M Abstract Quercetin is a polyphenolic flavonoid, which is frequently found in fruits and vegetables. The antioxidant potential of quercetin has been studied from subcellular compartments, that ... read more

Nucleic acid therapeutics in Huntington's disease. Recent Pat Biotechnol. 2019 Feb 08;: Authors: Singh K, Roy I Abstract BACKGROUND: Protein misfolding is a critical factor in the progression of a large number of neurodegenerative diseases. The incorrectly folded protein is prone to aggregation, leading in aberrant interaction with other cellular ... read more

Related ArticlesDefective Sphingosine-1-phosphate metabolism is a druggable target in Huntington's disease. Sci Rep. 2017 07 13;7(1):5280 Authors: Di Pardo A, Amico E, Basit A, Armirotti A, Joshi P, Neely MD, Vuono R, Castaldo S, Digilio AF, Scalabrì F, Pepe G, Elifani F, Madonna M, Jeong SK, Park BM, ... read more

Related ArticlesA Novel Therapy for Huntington's Disease. Cerebrum. 2018 Sep-Oct;2018: Authors: La Spada AR Abstract In 1979, while at the National Institutes of Health, now Columbia University professor Nancy Wexler and colleagues traveled to Venezuela to study the world's largest family with Huntington's disease. That led to identifying the disease ... read more

Related ArticlesA Quantitative Systems Pharmacology Approach to Infer Pathways Involved in Complex Disease Phenotypes. Methods Mol Biol. 2018;1787:207-222 Authors: Schurdak ME, Pei F, Lezon TR, Carlisle D, Friedlander R, Taylor DL, Stern AM Abstract Designing effective therapeutic strategies for complex diseases such as cancer and neurodegeneration that involve tissue ... read more

Related ArticlesImportance of the subcellular location of protein deposits in neurodegenerative diseases. Curr Opin Neurobiol. 2018 08;51:127-133 Authors: Bertolotti A Abstract Alzheimer's disease, Parkinson's, Huntington's, amyotrophic lateral sclerosis (ALS) and prion disorders are devastating neurodegenerative diseases of increasing prevalence in aging populations. Although clinically different, they share similar molecular ... read more

Protein misfolding and aggregation in neurodegenerative diseases: a review of pathogeneses, novel detection strategies, and potential therapeutics. Rev Neurosci. 2019 Feb 12;: Authors: Gandhi J, Antonelli AC, Afridi A, Vatsia S, Joshi G, Romanov V, Murray IVJ, Khan SA Abstract Protein folding is a complex, multisystem process characterized by heavy ... read more

Related ArticlesSfp1 links TORC1 and cell growth regulation to the yeast SAGA-complex component Tra1 in response to polyQ proteotoxicity. Traffic. 2019 Feb 10;: Authors: Jiang Y, Berg MD, Genereaux J, Ahmed K, Duennwald ML, Brandl CJ, Lajoie P Abstract Chromatin remodeling regulates gene expression in response to the accumulation of ... read more

Related ArticlesEndoplasmic Reticulum Stress: Implications for Neuropsychiatric Disorders. Chonnam Med J. 2019 Jan;55(1):8-19 Authors: Muneer A, Shamsher Khan RM Abstract The Endoplasmic reticulum (ER), an indispensable sub-cellular component of the eukaryotic cell carries out essential functions, is critical to the survival of the organism. The chaperone proteins and the folding ... read more

Related ArticlesMolecular insights into cortico-striatal miscommunications in Huntington's disease. Curr Opin Neurobiol. 2018 02;48:79-89 Authors: Veldman MB, Yang XW Abstract Huntington's disease (HD), a dominantly inherited neurodegenerative disease, is defined by its genetic cause, a CAG-repeat expansion in the HTT gene, its motor and psychiatric symptomology and primary ... read more

Tau and TDP-43 proteinopathies: kindred pathologic cascades and genetic pleiotropy. Lab Invest. 2019 Feb 11;: Authors: Chornenkyy Y, Fardo DW, Nelson PT Abstract We review the literature on Tau and TDP-43 proteinopathies in aged human brains and the relevant underlying pathogenetic cascades. Complex interacting pathways are implicated in Alzheimer's disease ... read more

Related ArticlesREST, a master transcriptional regulator in neurodegenerative disease. Curr Opin Neurobiol. 2018 02;48:193-200 Authors: Hwang JY, Zukin RS Abstract The restrictive element-1 silencing transcription factor)/NRSF (neuron-restrictive silencing factor (NRSF) is a transcriptional repressor which acts via epigenetic remodeling to silence target genes. Emerging evidence indicates that ... read more

Related ArticlesExtracellular vesicles in neurodegenerative diseases. Mol Aspects Med. 2018 04;60:52-61 Authors: Croese T, Furlan R Abstract Extracellular vesicles (EVs) are released by all neural cells, including neurons, oligodendrocytes, astrocytes, and microglia. The lack of adequate technology has not halted neuroscientists from investigating EVs as a mean to decipher ... read more

Related ArticlesSmall molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington's disease. Hum Mol Genet. 2018 05 01;27(9):1545-1555 Authors: Zhou X, Li G, Kaplan A, Gaschler MM, Zhang X, Hou Z, Jiang M, Zott R, Cremers S, ... read more

Related ArticlesWhen nature's robots go rogue: exploring protein homeostasis dysfunction and the implications for understanding human aging disease pathologies. Expert Rev Proteomics. 2018 04;15(4):293-309 Authors: Reisz JA, Barrett AS, Nemkov T, Hansen KC, D'Alessandro A Abstract INTRODUCTION: Proteins have been historically regarded as 'nature's robots': Molecular machines that are essential ... read more

Olfactory bulb atrophy and caspase activation observed in the BACHD rat models of Huntington disease. Neurobiol Dis. 2019 Feb 06;: Authors: Lessard-Beaudoin M, Yu-Taeger L, Laroche M, Singer E, Riess O, Nguyen HHP, Graham RK Abstract Olfactory dysfunction is observed in several neurological disorders, including Huntington disease (HD), and correlates ... read more

Related ArticlesA Microglial Signature Directing Human Aging and Neurodegeneration-Related Gene Networks. Front Neurosci. 2019;13:2 Authors: Mukherjee S, Klaus C, Pricop-Jeckstadt M, Miller JA, Struebing FL Abstract Aging is regarded as a major risk factor for neurodegenerative diseases. Thus, a better understanding of the similarities between the aging process and neurodegenerative ... read more

Related ArticlesTreatment of Tardive Dyskinesia: A General Overview with Focus on the Vesicular Monoamine Transporter 2 Inhibitors. Drugs. 2018 04;78(5):525-541 Authors: Niemann N, Jankovic J Abstract Tardive dyskinesia (TD) encompasses the spectrum of iatrogenic hyperkinetic movement disorders following exposure to dopamine receptor-blocking agents (DRBAs). Despite the advent of atypical ... read more

Enhanced striatopallidal gamma-aminobutyric acid (GABA)A receptor transmission in mouse models of huntington's disease. Mov Disord. 2019 Feb 06;: Authors: Perez-Rosello T, Gelman S, Tombaugh G, Cachope R, Beaumont V, James Surmeier D Abstract BACKGROUND: Huntington's disease (HD) is caused by a CAG repeat expansion in the huntingtin gene. This mutation ... read more

Excess active P13K rescues huntingtin-mediated neuronal cell death but has no effect on axonal transport defects. Apoptosis. 2019 Feb 06;: Authors: Hansen T, Thant C, White JA, Banerjee R, Thuamsang B, Gunawardena S Abstract High levels of oxidative stress is detected in neurons affected by many neurodegenerative diseases, including huntington's ... read more

Related ArticlesNascent-protein ubiquitination is required for heat shock-induced gene downregulation in human cells. Nat Struct Mol Biol. 2019 Feb;26(2):137-146 Authors: Aprile-Garcia F, Tomar P, Hummel B, Khavaran A, Sawarkar R Abstract Proteotoxic stress such as heat shock causes heat-shock factor (HSF)-dependent transcriptional upregulation of chaperones. Heat shock also leads to ... read more

Related ArticlesSingular Location and Signaling Profile of Adenosine A2A-Cannabinoid CB1 Receptor Heteromers in the Dorsal Striatum. Neuropsychopharmacology. 2018 04;43(5):964-977 Authors: Moreno E, Chiarlone A, Medrano M, Puigdellívol M, Bibic L, Howell LA, Resel E, Puente N, Casarejos MJ, Perucho J, Botta J, Suelves N, Ciruela F, Ginés S, ... read more

Related ArticlesHDNetDB: A Molecular Interaction Database for Network-Oriented Investigations into Huntington's Disease. Sci Rep. 2017 07 12;7(1):5216 Authors: Kalathur RKR, Pedro Pinto J, Sahoo B, Chaurasia G, Futschik ME Abstract Huntington's disease (HD) is a progressive and fatal neurodegenerative disorder caused by an expanded CAG repeat in the ... read more

StatPearls Book. 2018 01 Authors: Abstract The basal ganglia is a cluster of nuclei found deep to the neocortex of the brain. It has a multitude of functions associated with reward and cognition but is primarily involved in motor control. In particular, the basal ganglia is considered to be ... read more

Related ArticlesThe 17-residue-long N terminus in huntingtin controls stepwise aggregation in solution and on membranes via different mechanisms. J Biol Chem. 2018 02 16;293(7):2597-2605 Authors: Pandey NK, Isas JM, Rawat A, Lee RV, Langen J, Pandey P, Langen R Abstract Aggregation of huntingtin protein arising from expanded polyglutamine (polyQ) ... read more

Related ArticlesSmall interfering RNAs based on huntingtin trinucleotide repeats are highly toxic to cancer cells. EMBO Rep. 2018 03;19(3): Authors: Murmann AE, Gao QQ, Putzbach WE, Patel M, Bartom ET, Law CY, Bridgeman B, Chen S, McMahon KM, Thaxton CS, Peter ME Abstract Trinucleotide repeat (TNR) expansions in the ... read more

Transspinal delivery of drugs by transdermal patch back-of-neck for Alzheimer's disease: a new route of administration. Discov Med. 2019 Jan;27(146):37-43 Authors: Lehrer S, Rheinstein PH Abstract NSAIDs may prevent Alzheimer's disease (AD) but have failed as a treatment, possibly because only 1-2% of an oral NSAID dose reaches the brain. ... read more

Co-expression Network Analysis Revealing the Potential Regulatory Roles of lncRNAs in Alzheimer's Disease. Interdiscip Sci. 2019 Feb 04;: Authors: Wu J, Chen L, Zheng C, Xu S, Gao Y, Wang J Abstract Alzheimer's disease (AD) is one of the most common types of dementia among the elderly. Previous studies had ... read more