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Related ArticlesTETRABENAZINE FACILITATES EXOCYTOSIS BY ENHANCING CALCIUM-INDUCED CALCIUM RELEASE THROUGH RYANODINE RECEPTORS. J Pharmacol Exp Ther. 2019 Jun 17;: Authors: DE Pascual R, Alvarez-Ortego N, de Los Rios C, Jacob-Mazariego G, Garcia AG Abstract Vesicular monoamine transporter-2 (VMAT2) is expressed in the presynaptic secretory vesicles membrane in brain. Its blockade ... read more

Phenotype-genotype discrepancies in the prospective Huntington at-risk observational study. Ann Clin Transl Neurol. 2019 Jun;6(6):1046-1052 Authors: Shoulson I, Eberly S, Oakes D, Kayson E, Young AB, PHAROS Investigators Abstract Objective: To examine phenotype-genotype discrepancies (PGDs) wherein genotype-concealed and prospective judgments of the motor onset of Huntington disease (HD) occurred among ... read more

Related ArticlesThe Biosynthesis, Signaling, and Neurological Functions of Bile Acids. Biomolecules. 2019 Jun 15;9(6): Authors: Kiriyama Y, Nochi H Abstract Bile acids (BA) are amphipathic steroid acids synthesized from cholesterol in the liver. They act as detergents to expedite the digestion and absorption of dietary lipids and lipophilic vitamins. BA ... read more

Related ArticlesBrain Regions Showing White Matter Loss in Huntington's Disease Are Enriched for Synaptic and Metabolic Genes. Biol Psychiatry. 2018 03 01;83(5):456-465 Authors: McColgan P, Gregory S, Seunarine KK, Razi A, Papoutsi M, Johnson E, Durr A, Roos RAC, Leavitt BR, Holmans P, Scahill RI, Clark CA, Rees ... read more

Related ArticlesIntranasal Administration of Mesenchymal Stem Cells Ameliorates the Abnormal Dopamine Transmission System and Inflammatory Reaction in the R6/2 Mouse Model of Huntington Disease. Cells. 2019 Jun 15;8(6): Authors: Yu-Taeger L, Stricker-Shaver J, Arnold K, Bambynek-Dziuk P, Novati A, Singer E, Lourhmati A, Fabian C, Magg J, Riess O, Schwab ... read more

Related ArticlesPeroxisome Proliferator-Activated Receptor Gamma Coactivator-1 Alpha as a Novel Target for Bipolar Disorder and Other Neuropsychiatric Disorders. Biol Psychiatry. 2018 05 01;83(9):761-769 Authors: Nierenberg AA, Ghaznavi SA, Sande Mathias I, Ellard KK, Janos JA, Sylvia LG Abstract Peroxisome proliferator-activated receptor gamma coactivator-1 alpha (PGC-1 alpha) is a protein ... read more

Diagnostic Value of Cerebrospinal Fluid Neurofilament Light Protein in Neurology: A Systematic Review and Meta-analysis. JAMA Neurol. 2019 Jun 17;: Authors: Bridel C, van Wieringen WN, Zetterberg H, Tijms BM, Teunissen CE, and the NFL Group, Alvarez-Cermeño JC, Andreasson U, Axelsson M, Bäckström DC, Bartos A, Bjerke M, Blennow K, ... read more

Use of large animal models to investigate Huntington's diseases. Cell Regen (Lond). 2019 Jun;8(1):9-11 Authors: Yan S, Li S, Li XJ Abstract Animal models that can mimic human diseases are the important tools for investigating the pathogenesis of the diseases and finding a way for treatment. There is no doubt ... read more

Related ArticlesConformational studies of pathogenic expanded polyglutamine protein deposits from Huntington's disease. Exp Biol Med (Maywood). 2019 Jun 15;:1535370219856620 Authors: Matlahov I, van der Wel PC PMID: 31203656 [PubMed - as supplied by publisher] ... read more

Related ArticlesHuman DnaJB6 Antiamyloid Chaperone Protects Yeast from Polyglutamine Toxicity Separately from Spatial Segregation of Aggregates. Mol Cell Biol. 2018 12 01;38(23): Authors: Kumar J, Kline NL, Masison DC Abstract Polyglutamine (polyQ) aggregates are associated with pathology in protein-folding diseases and with toxicity in the yeast Saccharomyces cerevisiae ... read more

Uses for humanised mouse models in precision medicine for neurodegenerative disease. Mamm Genome. 2019 Jun 15;: Authors: Nair RR, Corrochano S, Gasco S, Tibbit C, Thompson D, Maduro C, Ali Z, Fratta P, Arozena AA, Cunningham TJ, Fisher EMC Abstract Neurodegenerative disease encompasses a wide range of disorders afflicting the ... read more

Phase separation in biology and disease-a symposium report. Ann N Y Acad Sci. 2019 Jun 14;: Authors: Cable J, Brangwynne C, Seydoux G, Cowburn D, Pappu RV, Castañeda CA, Berchowitz LE, Chen Z, Jonikas M, Dernburg A, Mittag T, Fawzi NL Abstract Phase separation of multivalent protein and RNA molecules ... read more

Meaningful and Measurable Health Domains in Huntington's Disease: Large-Scale Validation of the Huntington's Disease Health-Related Quality of Life Questionnaire Across Severity Stages. Value Health. 2019 Jun;22(6):712-720 Authors: Ho AK, Horton MC, Landwehrmeyer GB, Burgunder JM, Tennant A, European Huntington’s Disease Network Abstract BACKGROUND: Although health-related quality of life is key ... read more

Related ArticlesMetalloproteinases and their tissue inhibitors in Alzheimer's disease and other neurodegenerative disorders. Cell Mol Life Sci. 2019 Jun 13;: Authors: Rivera S, García-González L, Khrestchatisky M, Baranger K Abstract As life expectancy increases worldwide, age-related neurodegenerative diseases will increase in parallel. The lack of effective treatment strategies may soon lead to an ... read more

Related ArticlesClustering of longitudinal data by using an extended baseline: A new method for treatment efficacy clustering in longitudinal data. Stat Methods Med Res. 2018 01;27(1):97-113 Authors: Schramm C, Vial C, Bachoud-Lévi AC, Katsahian S Abstract Heterogeneity in treatment efficacy is a major concern in clinical trials. Clustering may ... read more

Related ArticlesMeasuring attitudes towards the dying process: A systematic review of tools. Palliat Med. 2018 04;32(4):815-837 Authors: Groebe B, Strupp J, Eisenmann Y, Schmidt H, Schlomann A, Rietz C, Voltz R Abstract BACKGROUND: At the end of life, anxious attitudes concerning the dying process are common in patients in ... read more

Related ArticlesThe views of adults with Huntington's disease on assisted dying: A qualitative exploration. Palliat Med. 2018 04;32(4):708-715 Authors: Regan L, Preston NJ, Eccles FJR, Simpson J Abstract BACKGROUND: Assisted dying is frequently debated publicly and research often includes the views of health professionals on this issue. However, the ... read more

Related ArticlesIntercellular Spread of Protein Aggregates in Neurodegenerative Disease. Annu Rev Cell Dev Biol. 2018 10 06;34:545-568 Authors: Davis AA, Leyns CEG, Holtzman DM Abstract Most neurodegenerative diseases are characterized by the accumulation of protein aggregates, some of which are toxic to cells. Mounting evidence demonstrates that in several ... read more

Profiling the genome-wide landscape of tandem repeat expansions. Nucleic Acids Res. 2019 Jun 13;: Authors: Mousavi N, Shleizer-Burko S, Yanicky R, Gymrek M Abstract Tandem repeat (TR) expansions have been implicated in dozens of genetic diseases, including Huntington's Disease, Fragile X Syndrome, and hereditary ataxias. Furthermore, TRs have recently been ... read more

Related ArticlesEnd-of-life measures in Huntington disease: HDQLIFE Meaning and Purpose, Concern with Death and Dying, and End of Life Planning. J Neurol. 2019 Jun 12;: Authors: Carlozzi NE, Boileau NR, Paulsen JS, Perlmutter JS, Lai JS, Hahn EA, McCormack MK, Nance MA, Cella D, Barton SK, Downing NR Abstract BACKGROUND AND PURPOSE: ... read more

Counter effects of Asiaticosids-D through putative neurotransmission on rotenone induced cerebral ganglionic injury in Lumbricus terrestris. IBRO Rep. 2019 Jun;6:160-175 Authors: Subaraja M, Vanisree AJ Abstract Asiaticoside-D (AD) was shown to efficacy of ganglionic degenerated Lumbricus terrestris as a pioneering observation in our earlier research. Though, extract molecular mechanisms of ... read more

Related ArticlesBrain iron transport. Biol Rev Camb Philos Soc. 2019 Jun 12;: Authors: Qian ZM, Ke Y Abstract Brain iron is a crucial participant and regulator of normal physiological activity. However, excess iron is involved in the formation of free radicals, and has been associated with oxidative damage to neuronal ... read more

Related ArticlesClosing-in Phenomenon in Huntington's Disease: A Neuropsychological Marker of Frontal/Executive Dysfunction. Arch Clin Neuropsychol. 2019 Feb 01;34(1):24-30 Authors: De Lucia N, Peluso S, Roca A, Russo CV, Massarelli M, De Michele G, Di Maio L, Salvatore E, De Michele G Abstract Objective: In visuo-constructional tasks, patients may reproduce ... read more

Dual Role of Autophagy in Diseases of the Central Nervous System. Front Cell Neurosci. 2019;13:196 Authors: Bar-Yosef T, Damri O, Agam G Abstract Autophagy is a vital lysosomal degradation and recycling pathway in the eukaryotic cell, responsible for maintaining an intricate balance between cell survival and cell death, necessary for ... read more

Related ArticlesSelected health and lifestyle factors, cytosine-adenine-guanine status, and phenoconversion in Huntington's disease. Mov Disord. 2018 03;33(3):472-478 Authors: Tanner C, Marder K, Eberly S, Biglan K, Oakes D, Shoulson I, Huntington Study Group Prospective Huntington At-Risk Observational Study Investigators Abstract BACKGROUND: In Huntington's disease, 60% of the variance ... read more

Related ArticlesThe influence of motor ability rehabilitation on temporal-spatial parameters of gait in Huntington's disease patients on the basis of a three-dimensional motion analysis system: An experimental trial. Neurol Neurochir Pol. 2018 Sep - Oct;52(5):575-580 Authors: Mirek E, Filip M, Chwała W, Szymura J, Pasiut S, Banaszkiewicz K, Bar MR, ... read more

Related ArticlesMotor cortex synchronization influences the rhythm of motor performance in premanifest huntington's disease. Mov Disord. 2018 03;33(3):440-448 Authors: Casula EP, Mayer IMS, Desikan M, Tabrizi SJ, Rothwell JC, Orth M Abstract BACKGROUND: In Huntington's disease there is evidence of structural damage in the motor system, but it is ... read more

Related ArticlesReducing Mutant Huntingtin Protein Expression in Living Cells by a Newly Identified RNA CAG Binder. ACS Chem Neurosci. 2018 06 20;9(6):1399-1408 Authors: Matthes F, Massari S, Bochicchio A, Schorpp K, Schilling J, Weber S, Offermann N, Desantis J, Wanker E, Carloni P, Hadian K, Tabarrini O, Rossetti ... read more

Related ArticlesDetermination of Parameters of Oxidative Stress in vitro Models of Neurodegenerative Diseases-A Review. Curr Clin Pharmacol. 2018;13(2):100-109 Authors: Feitosa CM, da Silva Oliveira GL, do Nascimento Cavalcante A, Morais Chaves SK, Rai M Abstract Oxidative stress is a major mechanism underlying the development of various neurodegenerative diseases ... read more

Comparative Transcriptome Analysis of Unusual Localized Skin Laxity in Sika Deer (Cervus nippon). DNA Cell Biol. 2019 Jun 12;: Authors: Chen X, Yang Y, Chang T, Xu B, Wei H Abstract Cutis laxa represents a heterogeneous group of rare, inherited, or acquired connective tissue disorders with the common feature of ... read more

Related ArticlesSatellite cell content in Huntington's disease patients in response to endurance training. Orphanet J Rare Dis. 2019 Jun 11;14(1):135 Authors: Mueller SM, Mihaylova V, Frese S, Petersen JA, Ligon-Auer M, Aguayo D, Flück M, Jung HH, Toigo M Abstract BACKGROUND: Skeletal muscle wasting is a hallmark of Huntington's disease ... read more

Network spread determines severity of degeneration and disconnection in Huntington's disease. Hum Brain Mapp. 2019 Jun 12;: Authors: Poudel GR, Harding IH, Egan GF, Georgiou-Karistianis N Abstract Trans-neuronal propagation of mutant huntingtin protein contributes to the organised spread of cortico-striatal degeneration and disconnection in Huntington's disease (HD). We investigated whether ... read more

Benefits of curcumin in brain disorders. Biofactors. 2019 Jun 11;: Authors: Bhat A, Mahalakshmi AM, Ray B, Tuladhar S, Hediyal TA, Manthiannem E, Padamati J, Chandra R, Chidambaram SB, Sakharkar MK Abstract Curcumin is widely consumed in Asia either as turmeric directly or as one of the culinary ingredients in ... read more

Lowering Mutant Huntingtin Using Tricyclo-DNA Antisense Oligonucleotides As a Therapeutic Approach for Huntington's Disease. Nucleic Acid Ther. 2019 Jun 11;: Authors: Imbert M, Blandel F, Leumann C, Garcia L, Goyenvalle A Abstract Huntington's disease is a neurodegenerative disorder caused by a CAG repeat expansion in the first exon of huntingtin ... read more

Related ArticlesEmerging therapies in Huntington's disease. Expert Rev Neurother. 2019 Jun 11;: Authors: Bashir H Abstract Introduction Huntington's disease (HD) is an inherited neurodegenerative condition for which there are no disease-modifying treatments. The availability of early genetic diagnosis makes HD an ideal candidate for early intervention. Growing understanding of pathogenesis ... read more

Related ArticlesAluminum in neurological disease - a 36 year multicenter study. J Alzheimers Dis Parkinsonism. 2019;8(6): Authors: Lukiw WJ, Kruck TPA, Percy ME, Pogue AI, Alexandrov PN, Walsh WJ, Sharfman NM, Jaber VR, Zhao Y, Li W, Bergeron C, Culicchia F, Fang Z, McLachlan DRC Abstract Aluminum is a ubiquitous ... read more

Ancillary Service Utilization and Impact in Huntington's Disease. J Huntingtons Dis. 2019 Jun 03;: Authors: Yomtoob J, Yeh C, Bega D Abstract BACKGROUND: Prior Huntington's disease (HD) studies suggest ancillary services improve motor symptoms, cognition, mood, and quality of life but frequency of use and clinicalcharacteristics are unclear. OBJECTIVE: Describe ... read more

Related ArticlesThe role of Nrf2 signaling in counteracting neurodegenerative diseases. FEBS J. 2018 10;285(19):3576-3590 Authors: Dinkova-Kostova AT, Kostov RV, Kazantsev AG Abstract The transcription factor Nrf2 (nuclear factor-erythroid 2 p45-related factor 2) functions at the interface of cellular redox and intermediary metabolism. Nrf2 target genes encode antioxidant enzymes, ... read more

Related ArticlesArtificial miRNAs Reduce Human Mutant Huntingtin Throughout the Striatum in a Transgenic Sheep Model of Huntington's Disease. Hum Gene Ther. 2018 06;29(6):663-673 Authors: Pfister EL, DiNardo N, Mondo E, Borel F, Conroy F, Fraser C, Gernoux G, Han X, Hu D, Johnson E, Kennington L, Liu P, Reid SJ, ... read more

Related ArticlesInositol 1,4,5-trisphosphate receptors and neurodegenerative disorders. FEBS J. 2018 10;285(19):3547-3565 Authors: Egorova PA, Bezprozvanny IB Abstract The inositol 1,4,5-trisphosphate receptor (IP3 R) is an intracellular ion channel that mediates the release of calcium ions from the endoplasmic reticulum. It plays a role in basic biological functions, such as ... read more

Related ArticlesCan leukocyte telomere shortening be a possible biomarker to track Huntington's disease progression? Neural Regen Res. 2019 Oct;14(10):1709-1710 Authors: Mantuano E, Peconi M, Scarabino D PMID: 31169183 [PubMed] ... read more

Related ArticlesGeorge Huntington (1850-1916). J Neurol. 2019 Mar;266(3):793-795 Authors: Owecki MK, Magowska A AbstractPMID: 29713824 [PubMed - indexed for MEDLINE] ... read more

Age-Related Oxidative Changes in Primary Porcine Fibroblasts Expressing Mutated Huntingtin. Neurodegener Dis. 2019 Jun 05;:1-13 Authors: Smatlikova P, Askeland G, Vaskovicova M, Klima J, Motlik J, Eide L, Ellederová Z Abstract BACKGROUND: Huntington's disease (HD) is a devastating neurodegenerative disorder caused by CAG triplet expansions in the huntingtin gene. Oxidative ... read more

Metformin treatment reduces motor and neuropsychiatric phenotypes in the zQ175 mouse model of Huntington disease. Exp Mol Med. 2019 Jun 05;51(6):65 Authors: Sanchis A, García-Gimeno MA, Cañada-Martínez AJ, Sequedo MD, Millán JM, Sanz P, Vázquez-Manrique RP Abstract Huntington disease is a neurodegenerative condition for which there is no cure to ... read more

Biocompatible inhibitor based on chitosan and amphiphilic peptide against mutant Huntingtin toxicity. Chembiochem. 2019 Jun 05;: Authors: Wahyuningtyas D, Chen WH, Huang CH, He YJ, Huang JJ Abstract Huntington's disease (HD) is classified as protein-misfolding diseases correlated with the mutant Huntingtin (mHtt) protein with abnormally expanded polyglutamine (polyQ) domains. Since ... read more

Use of human pluripotent stem cell-derived cells for neurodegenerative disease modeling and drug screening platform. Future Med Chem. 2019 Jun 04;: Authors: Garcia-Leon JA, Vitorica J, Gutierrez A Abstract Most neurodegenerative diseases are characterized by a complex and mostly still unresolved pathology. This fact, together with the lack of reliable ... read more

Impairment and Restoration of Homeostatic Plasticity in Cultured Cortical Neurons From a Mouse Model of Huntington Disease. Front Cell Neurosci. 2019;13:209 Authors: Smith-Dijak AI, Nassrallah WB, Zhang LYJ, Geva M, Hayden MR, Raymond LA Abstract Huntington disease (HD) is an inherited neurodegenerative disorder caused by a mutation in the huntingtin ... read more

Related ArticlesNucleocytoplasmic transport defects in neurodegeneration - cause or consequence? Semin Cell Dev Biol. 2019 May 29;: Authors: Hutten S, Dormann D Abstract Defects in nucleocytoplasmic transport have been associated with several neurodegenerative disorders and, in particular, the formation of pathological protein aggregates characteristic for the respective disease. However, whether ... read more

Related ArticlesLiterature Evidence and ARRIVE Assessment on Neuroprotective Effects of Flavonols in Neurodegenerative Diseases' Models. CNS Neurol Disord Drug Targets. 2018 04 26;17(1):34-42 Authors: Bombardi Duarte AC, Santana MG, di Camilo Orfali G, de Oliveira CTP, Priolli DG Abstract BACKGROUND AND OBJECTIVE: This paper was based on a ... read more

Related ArticlesMisdiagnosis of Huntington's disease. Lancet Psychiatry. 2017 01;4(1):21 Authors: Mustafa FA PMID: 28012477 [PubMed - indexed for MEDLINE] ... read more