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Related ArticlesDynasore Suppresses mTORC1 Activity and Induces Autophagy to Regulate the Clearance of Protein Aggregates in Neurodegenerative Diseases. Neurotox Res. 2019 Mar 29;: Authors: Chen Y, Xu S, Wang N, Ma Q, Peng P, Yu Y, Zhang L, Ying Z, Wang H Abstract Autophagy is an important cellular protein control ... read more

Related ArticlesTrojan triplets: RNA-based pathomechanisms for muscle dysfunction in Huntington's disease. J Gen Physiol. 2017 01;149(1):49-53 Authors: Skov M, Dirksen RT PMID: 27940949 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesAnalysis of 50 Neurodegenerative Genes in Clinically Diagnosed Early-Onset Alzheimer's Disease. Int J Mol Sci. 2019 Mar 26;20(6): Authors: Giau VV, Senanarong V, Bagyinszky E, An SSA, Kim S Abstract Alzheimer's disease (AD), Parkinson's disease (PD), frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), Huntington's disease (HD), and prion diseases ... read more

Application of an in vitro Blood Brain Barrier model in the Selection of Experimental Drug Candidates for the Treatment of Huntington's Disease. Mol Pharm. 2019 Mar 27;: Authors: Di Marco A, Gonzalez Paz O, Fini I, Vignone D, Cellucci A, Battista MR, Auciello G, Orsatti L, Zini M, Monteagudo E, ... read more

Related ArticlesNeurophysiological and Behavioral Effects of Anti-Orexinergic Treatments in a Mouse Model of Huntington's Disease. Neurotherapeutics. 2019 Mar 26;: Authors: Cabanas M, Pistono C, Puygrenier L, Rakesh D, Jeantet Y, Garret M, Cho YH Abstract Huntington's disease (HD) is associated with sleep and circadian disturbances in addition to hallmark motor ... read more

Related ArticlesATAD3A oligomerization causes neurodegeneration by coupling mitochondrial fragmentation and bioenergetics defects. Nat Commun. 2019 Mar 26;10(1):1371 Authors: Zhao Y, Sun X, Hu D, Prosdocimo DA, Hoppel C, Jain MK, Ramachandran R, Qi X Abstract Mitochondrial fragmentation and bioenergetic failure manifest in Huntington's disease (HD), a fatal neurodegenerative disease. The ... read more

Related ArticlesReconstituting Corticostriatal Network on-a-Chip Reveals the Contribution of the Presynaptic Compartment to Huntington's Disease. Cell Rep. 2018 01 02;22(1):110-122 Authors: Virlogeux A, Moutaux E, Christaller W, Genoux A, Bruyère J, Fino E, Charlot B, Cazorla M, Saudou F Abstract Huntington's disease (HD), a devastating neurodegenerative disorder, strongly affects ... read more

Related ArticlesAstroglial monoacylglycerol lipase controls mutant huntingtin-induced damage of striatal neurons. Neuropharmacology. 2019 Mar 23;: Authors: Ruiz-Calvo A, Bajo-Grañeras R, Maroto IB, Zian D, Grabner GF, García-Taboada E, Resel E, Zechner R, Zimmermann R, Ortega-Gutiérrez S, Galve-Roperh I, Bellocchio L, Guzmán M Abstract Cannabinoids exert neuroprotection in a wide array ... read more

Related ArticlesSafety, efficacy, and mechanisms of action of cannabinoids in neurological disorders. Lancet Neurol. 2019 Mar 22;: Authors: Friedman D, French JA, Maccarrone M Abstract In the past two decades, there has been an increasing interest in the therapeutic potential of cannabinoids for neurological disorders such as epilepsy, multiple sclerosis, ... read more

Meso scale discovery-based assays for the detection of aggregated huntingtin. PLoS One. 2019;14(3):e0213521 Authors: Reindl W, Baldo B, Schulz J, Janack I, Lindner I, Kleinschmidt M, Sedaghat Y, Thiede C, Tillack K, Schmidt C, Cardaun I, Schwagarus T, Herrmann F, Hotze M, Osborne GF, Herrmann S, Weiss A, Zerbinatti C, ... read more

Related Articles2,4 Dinitrophenol as Medicine. Cells. 2019 Mar 23;8(3): Authors: Geisler JG Abstract In the sanctity of pure drug discovery, objective reasoning can become clouded when pursuing ideas that appear unorthodox, but are spot on physiologically. To put this into historical perspective, it was an unorthodox idea in the 1950's ... read more

A Meta-Analysis of Alzheimer's Disease Brain Transcriptomic Data. J Alzheimers Dis. 2019 Mar 18;: Authors: Patel H, Dobson RJB, Newhouse SJ Abstract BACKGROUND: Microarray technologies have identified imbalances in the expression of specific genes and biological pathways in Alzheimer's disease (AD) brains. However, there is a lack of reproducibility across ... read more

Related ArticlesNovel proteomic changes in brain mitochondria provide insights into mitochondrial dysfunction in mouse models of Huntington's disease. Mitochondrion. 2019 Mar 19;: Authors: Agrawal S, Fox JH Abstract Huntington's disease (HD) is a progressive ultimately fatal disorder caused by a glutamine-encoding CAG expansion in the huntingtin (HTT) gene that results ... read more

Related ArticlesAlterations in GABAA-Receptor Trafficking and Synaptic Dysfunction in Brain Disorders. Front Cell Neurosci. 2019;13:77 Authors: Mele M, Costa RO, Duarte CB Abstract GABAA receptors (GABAAR) are the major players in fast inhibitory neurotransmission in the central nervous system (CNS). Regulation of GABAAR trafficking and the control of their surface ... read more

Related ArticlesUsing induced pluripotent stem cell neuronal models to study neurodegenerative diseases. Biochim Biophys Acta Mol Basis Dis. 2019 Mar 18;: Authors: Zhang X, Hu D, Shang Y, Qi X Abstract Current application of human induced pluripotent stem cells (hiPSCs) technology in patient-specific models of neurodegenerative disorders recapitulate some of ... read more

Hypomorphic mutation of the mouse Huntington's disease gene orthologue. PLoS Genet. 2019 Mar 21;15(3):e1007765 Authors: Murthy V, Tebaldi T, Yoshida T, Erdin S, Calzonetti T, Vijayvargia R, Tripathi T, Kerschbamer E, Seong IS, Quattrone A, Talkowski ME, Gusella JF, Georgopoulos K, MacDonald ME, Biagioli M Abstract Rare individuals with inactivating ... read more

Amelioration of Huntington's disease phenotype in astrocytes derived from iPSC-derived neural progenitor cells of Huntington's disease monkeys. PLoS One. 2019;14(3):e0214156 Authors: Cho IK, Yang B, Forest C, Qian L, Chan AWS Abstract Huntington's disease (HD) is a devastating monogenic, dominant, hereditary, neurodegenerative disease. HD is caused by the expansion of ... read more

Related ArticlesInterrelationships Among Gut Microbiota and Host: Paradigms, Role in Neurodegenerative Diseases and Future Prospects. CNS Neurol Disord Drug Targets. 2017;16(8):945-964 Authors: Caballero-Villarraso J, Galvan A, Escribano BM, Tunez I Abstract BACKGROUND & OBJECTIVE: Advances in the knowledge of the microbiota and concepts related to it have triggered a ... read more

Related ArticlesFluorescence Imaging of Huntingtin mRNA Knockdown. Bioconjug Chem. 2018 04 18;29(4):1276-1282 Authors: Oh E, Liu Y, Sonar MV, Merry DE, Wickstrom E Abstract Huntington's disease (HD) is an autosomal-dominant neurodegenerative genetic disorder caused by CAG repeat expansion in exon 1 of the HTT gene. Expression of the mutant ... read more

The Step Test Evaluation of Performance on Stairs (STEPS): Validation and reliability in a neurological disorder. PLoS One. 2019;14(3):e0213698 Authors: Kloos AD, Kegelmeyer DA, Ambrogi K, Kline D, McCormack-Mager M, Schroeder B, Kostyk SK Abstract BACKGROUND: Individuals with neurological disorders often have difficulty negotiating stairs that can lead to injurious ... read more

Related ArticlesNeural Stem Cells and Human Induced Pluripotent Stem Cells to Model Rare CNS Diseases. CNS Neurol Disord Drug Targets. 2017;16(8):915-926 Authors: De Filippis L, Zalfa C, Ferrari D Abstract BACKGROUND & OBJECTIVE: Despite the great effort spent over recent decades to unravel the pathological mechanisms underpinning the ... read more

Related ArticlesImmunomodulatory Strategies for Huntington's Disease Treatment. CNS Neurol Disord Drug Targets. 2017;16(8):936-944 Authors: Colpo GD, Rocha NP, Stimming EF, Teixeira AL Abstract BACKGROUND & OBJECTIVE: Huntington's disease (HD) is an autosomal-dominant, progressive neurodegenerative disease characterized by selective loss of neurons in the striatum and cortex, which leads ... read more

Related ArticlesDiscovery of Small Molecules that Induce the Degradation of Huntingtin. Angew Chem Int Ed Engl. 2017 09 11;56(38):11530-11533 Authors: Tomoshige S, Nomura S, Ohgane K, Hashimoto Y, Ishikawa M Abstract Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by the aggregation of mutant huntingtin (mHtt), and ... read more

Related ArticlesA case-note review of continued pregnancies found to be at a high risk of Huntington's disease: considerations for clinical practice. Eur J Hum Genet. 2019 Mar 19;: Authors: Wadrup F, Holden S, MacLeod R, Miedzybrodzka Z, Németh AH, Owens S, Pasalodos S, Quarrell O, Clarke AJ, UK Huntington’s Disease ... read more

Related ArticlesA Huntingtin Knockin Pig Model Recapitulates Features of Selective Neurodegeneration in Huntington's Disease. Cell. 2018 05 03;173(4):989-1002.e13 Authors: Yan S, Tu Z, Liu Z, Fan N, Yang H, Yang S, Yang W, Zhao Y, Ouyang Z, Lai C, Yang H, Li L, Liu Q, Shi H, Xu G, ... read more

Related ArticlesEfficacy of ethyl-EPA as a treatment for Huntington disease: A systematic review and meta-analysis. Acta Neuropsychiatr. 2019 Mar 20;:1-38 Authors: Morsy S, Khalil SM, Doheim MF, Kamel MG, El-Basiony DSM, Hassan HIA, Eisa AA, Ngoc CTA, Dang NP, Hirayama K, Huy NT Abstract OBJECTIVE: After MRI studies suggested the ... read more

Related ArticlesA Drosophila model of Huntington disease-like 2 exhibits nuclear toxicity and distinct pathogenic mechanisms from Huntington disease. Hum Mol Genet. 2016 08 01;25(15):3164-3177 Authors: Krench M, Cho RW, Littleton JT Abstract Huntington disease-like 2 (HDL2) and Huntington disease (HD) are adult-onset neurodegenerative diseases characterized by movement disorders, ... read more

Related ArticlesSkeletal muscle characteristics and mitochondrial function in Huntington's disease patients. Mov Disord. 2017 08;32(8):1258-1259 Authors: Gehrig SM, Petersen JA, Frese S, Mueller SM, Mihaylova V, Ligon-Auer M, Lundby C, Toigo M, Jung HH PMID: 28620963 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesA hydrocortisone derivative binds to GAPDH and reduces the toxicity of extracellular polyglutamine-containing aggregates. Biochem Biophys Res Commun. 2017 Jun 03;487(3):723-727 Authors: Lazarev VF, Mikhaylova ER, Dutysheva EA, Suezov RV, Guzhova IV, Margulis BA Abstract Huntington's disease (HD) has been recently shown to have a horizontally transmitted, prion-like ... read more

Related ArticlesInclusions of R6/2 Mice Are Not Amyloid and Differ Structurally from Those of Huntington Disease Brain. Anal Chem. 2017 05 16;89(10):5201-5209 Authors: André W, Sandt C, Nondier I, Djian P, Hoffner G Abstract R6/2 mice contain an N-terminal fragment of human huntingtin with an expanded polyQ and develop ... read more

Related ArticlesEffect of Apolipoprotein E Genotypes on Huntington's Disease Phenotypes in a Han Chinese Population. Neurosci Bull. 2019 Mar 18;: Authors: Li XY, Zhang YB, Xu M, Cheng HR, Dong Y, Ni W, Li HL, Wu ZY Abstract Huntington's disease (HD) is an autosomal dominant degenerative disease that mainly encompasses ... read more

Related ArticlesTRIAD3/RNF216 E3 ligase specifically synthesises K63-linked ubiquitin chains and is inactivated by mutations associated with Gordon Holmes syndrome. Cell Death Discov. 2019;5:75 Authors: Schwintzer L, Aguado Roca E, Broemer M Abstract TRIAD3/RNF216 is a ubiquitin ligase of the RING-in-between-RING family. Recent publications identified TRIAD3 mutations in patients with neurological ... read more

The prevalence and the burden of pain in patients with Huntington disease: a systematic review and meta-analysis. Pain. 2019 Apr;160(4):773-783 Authors: Sprenger GP, van der Zwaan KF, Roos RAC, Achterberg WP Abstract It is remarkable that studies focusing on the prevalence and the burden of pain in patients with Huntington ... read more

Related ArticlesTherapeutic Inhibition of the Complement System in Diseases of the Central Nervous System. Front Immunol. 2019;10:362 Authors: Carpanini SM, Torvell M, Morgan BP Abstract The complement system plays critical roles in development, homeostasis, and regeneration in the central nervous system (CNS) throughout life; however, complement dysregulation in the CNS ... read more

Related ArticlesFXTAS: regional decrease of mitochondrial DNA copy number relates to clinical manifestations. Genes Brain Behav. 2019 Mar 18;:e12565 Authors: Alvarez-Mora MI, Podlesniy P, Gelpi E, Hukema R, Madrigal I, Pagonabarraga J, Trullas R, Mila M, Rodriguez-Revenga L Abstract Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late-onset neurodegenerative disorder that ... read more

Related ArticlesA protein quality control pathway regulated by linear ubiquitination. EMBO J. 2019 Mar 18;: Authors: van Well EM, Bader V, Patra M, Sánchez-Vicente A, Meschede J, Furthmann N, Schnack C, Blusch A, Longworth J, Petrasch-Parwez E, Mori K, Arzberger T, Trümbach D, Angersbach L, Showkat C, Sehr DA, Berlemann ... read more

Related ArticlesPresymptomatic testing of those at 25% risk of autosomal dominant neurodegenerative disease- testing team beware. Eur J Hum Genet. 2019 01;27(1):20-21 Authors: Delatycki MB AbstractPMID: 30291342 [PubMed - indexed for MEDLINE] ... read more

The effects of huntingtin-lowering: what do we know so far? Degener Neurol Neuromuscul Dis. 2019;9:3-17 Authors: Kaemmerer WF, Grondin RC Abstract Therapies targeting mutant huntingtin DNA, mRNA, and protein have a chance at becoming the first disease-modifying treatments for Huntington's disease, a fatal inherited neurodegenerative disorder for which only symptom ... read more

Modulation of Phospho-CREB by Systemically Administered Recombinant BDNF in the Hippocampus of the R6/2 Mouse Model of Huntington's Disease. Neurosci J. 2019;2019:8363274 Authors: Paldino E, Giampà C, Montagna E, Angeloni C, Fusco FR Abstract Huntington's disease (HD) is an autosomal dominant neurodegenerative disease due to an expansion of a trinucleotide ... read more

Related ArticlesPatient-reported outcome measures in Huntington disease: Quality of life in neurological disorders (Neuro-QoL) social functioning measures. Psychol Assess. 2018 04;30(4):450-458 Authors: Carlozzi NE, Hahn EA, Goodnight SM, Kratz AL, Paulsen JS, Stout JC, Frank S, Miner JA, Cella D, Gershon RC, Schilling SG, Ready RE Abstract Social functioning ... read more

Related ArticlesFunctional Differences between Synaptic Mitochondria from the Striatum and the Cerebral Cortex. Neuroscience. 2019 Mar 12;: Authors: Petersen MH, Willert CW, Andersen JV, Waagepetersen HS, Skotte NH, Nørremølle A Abstract Mitochondrial dysfunction has been shown to play a major role in neurodegenerative disorders such as Huntington's disease, Alzheimer's disease ... read more

Related ArticlesSmall molecules as therapeutic drugs for Alzheimer's disease. Mol Cell Neurosci. 2019 Mar 12;: Authors: Oliver DMA, Reddy PH Abstract Mitochondrial dysfunction is a central protagonist of Alzheimer's disease (AD) pathogenesis. Mitochondrial dysfunction stems from various factors including mitochondrial DNA damage and oxidative stress from reactive oxygen species, membrane ... read more

Related ArticlesThe therapeutic role of minocycline in Parkinson's disease. Drugs Context. 2019;8:212553 Authors: Cankaya S, Cankaya B, Kilic U, Kilic E, Yulug B Abstract Minocycline, a semisynthetic tetracycline-derived antibiotic, has been shown to exert anti-apoptotic, anti-inflammatory, and antioxidant effects. Furthermore, there is rapidly growing evidence suggesting that minocycline may have ... read more

Suicidal ideation and behavior in Huntington's disease: Systematic review and recommendations. J Affect Disord. 2019 Mar 05;250:319-329 Authors: Kachian ZR, Cohen-Zimerman S, Bega D, Gordon B, Grafman J Abstract BACKGROUND: An increased risk of suicide in Huntington's disease (HD) patients is well documented, with rates significantly higher than those of ... read more

Related Articles[Psychiatric symptoms in patients with Huntington's disease]. Ugeskr Laeger. 2018 Mar 26;180(13): Authors: Jensen RN, Bolwig T, Sørensen SA Abstract Huntington's disease is an inherited neuropsychiatric disorder characterized by a triad of symptoms: motor, cognitive and psychiatric. Psychiatric symptoms occur prior to the motor symptoms in approximately 50% ... read more

Related ArticlesHR23B pathology preferentially co-localizes with p62, pTDP-43 and poly-GA in C9ORF72-linked frontotemporal dementia and amyotrophic lateral sclerosis. Acta Neuropathol Commun. 2019 Mar 13;7(1):39 Authors: Riemslagh FW, Lans H, Seelaar H, Severijnen LWFM, Melhem S, Vermeulen W, Aronica E, Pasterkamp RJ, van Swieten JC, Willemsen R Abstract Human homologue of ... read more

Related ArticlesHuntington's disease in the United States: Variation by demographic and socioeconomic factors. Mov Disord. 2019 Mar 13;: Authors: Bruzelius E, Scarpa J, Zhao Y, Basu S, Faghmous JH, Baum A Abstract BACKGROUND: Despite extensive research regarding the etiology of Huntington's disease, relatively little is known about the epidemiology of ... read more

Related ArticlesDoes N-terminal huntingtin function as a 'holdase' for inhibiting cellular protein aggregation? FEBS J. 2018 05;285(10):1791-1811 Authors: Sethi R, Tripathi N, Pallapati AR, Gaikar A, Bharatam PV, Roy I Abstract Proteolytic cleavage of huntingtin gives rise to N-terminal fragments. While the role of truncated mutant huntingtin is described ... read more

Related ArticlesClinical manifestations of homozygote allele carriers in Huntington disease. Neurology. 2019 Mar 13;: Authors: Cubo E, Martinez-Horta SI, Santalo FS, Descalls AM, Calvo S, Gil-Polo C, Muñoz I, Llano K, Mariscal N, Diaz D, Gutierrez A, Aguado L, Ramos-Arroyo MA, European HD Network Abstract OBJECTIVE: Because patients homozygous for ... read more

Related ArticlesAlterations in the tyrosine and phenylalanine pathways revealed by biochemical profiling in cerebrospinal fluid of Huntington's disease subjects. Sci Rep. 2019 Mar 11;9(1):4129 Authors: Herman S, Niemelä V, Emami Khoonsari P, Sundblom J, Burman J, Landtblom AM, Spjuth O, Nyholm D, Kultima K Abstract Huntington's disease (HD) is a ... read more