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Related ArticlesMajor Contribution of Somatostatin-Expressing Interneurons and Cannabinoid Receptors to Increased GABA Synaptic Activity in the Striatum of Huntington's Disease Mice. Front Synaptic Neurosci. 2019;11:14 Authors: Holley SM, Galvan L, Kamdjou T, Dong A, Levine MS, Cepeda C Abstract Huntington's disease (HD) is a heritable neurological disorder that affects cognitive ... read more

Related ArticlesGlobal Proteome and Ubiquitinome Changes in the Soluble and Insoluble Fractions of Q175 Huntington Mice Brains. Mol Cell Proteomics. 2019 May 28;: Authors: Sap KA, Guler AT, Bezstarosti K, Bury AE, Juenemann K, Demmers J, Reits E Abstract Huntington's disease is caused by a polyglutamine repeat expansion in the ... read more

Related ArticlesInflammation in CNS neurodegenerative diseases. Immunology. 2018 06;154(2):204-219 Authors: Stephenson J, Nutma E, van der Valk P, Amor S Abstract Neurodegenerative diseases, the leading cause of morbidity and disability, are gaining increased attention as they impose a considerable socioeconomic impact, due in part to the ageing community. Neuronal ... read more

Related ArticlesWhole-Brain Connectivity in a Large Study of Huntington's Disease Gene Mutation Carriers and Healthy Controls. Brain Connect. 2018 04;8(3):166-178 Authors: Espinoza FA, Turner JA, Vergara VM, Miller RL, Mennigen E, Liu J, Misiura MB, Ciarochi J, Johnson HJ, Long JD, Bockholt HJ, Magnotta VA, Paulsen JS, Calhoun VD ... read more

Related ArticlesThe molecular epidemiology of Huntington disease is related to intermediate allele frequency and haplotype in the general population. Am J Med Genet B Neuropsychiatr Genet. 2018 04;177(3):346-357 Authors: Kay C, Collins JA, Wright GEB, Baine F, Miedzybrodzka Z, Aminkeng F, Semaka AJ, McDonald C, Davidson M, Madore SJ, ... read more

Related ArticlesA comprehensive in silico analysis of huntingtin and its interactome. J Biomol Struct Dyn. 2018 Sep;36(12):3155-3171 Authors: Brandi V, Di Lella V, Marino M, Ascenzi P, Polticelli F Abstract A polyglutamine expansion of the N-terminal region of huntingtin (Htt) causes Huntington's disease, a severe neurodegenerative disorder. Htt huge multidomain ... read more

Related ArticlesGolgi Complex Dynamics and Its Implication in Prevalent Neurological Disorders. Front Cell Dev Biol. 2019;7:75 Authors: Caracci MO, Fuentealba LM, Marzolo MP Abstract Coupling of protein synthesis with protein delivery to distinct subcellular domains is essential for maintaining cellular homeostasis, and defects thereof have consistently been shown to be ... read more

Related ArticlesProtein misfolding, aggregation and mechanism of amyloid cytotoxicity: An overview and therapeutic strategies to inhibit aggregation. Int J Biol Macromol. 2019 May 22;: Authors: Zaman M, Khan AN, Wahiduzzaman, Zakariya SM, Khan RH Abstract Protein and peptides are converted from their soluble forms into highly ordered fibrillar aggregates under ... read more

Related ArticlesNucleus-cytoplasm cross-talk in the aging brain. J Neurosci Res. 2019 May 26;: Authors: Benvegnù S Abstract Aging is a primary risk factor for fatal neurodegenerative disorders, yet the mechanisms underlying physiological healthy aging and pathological aging, and how these mechanisms can divert one scenario to the other, are not ... read more

Related ArticlesAn antibiotic (sulfamethoxazole) stabilizes polypeptide (human serum albumin) even under extreme condition (elevated temperature). Int J Biol Macromol. 2019 May 22;: Authors: Furkan M, Sidddiqi K, Khan AN, Khan RH Abstract Since aggregation of protein result into number of human diseases including diabetes mellitus, Huntington's and Alzheimer's disease, etc. ... read more

Related ArticlesIdentification of novel allosteric modulators of mGlu5 acting at site distinct from MPEP binding. ACS Chem Neurosci. 2019 May 27;: Authors: Butkiewicz M, Rodriguez AL, Rainey SE, Wieting JM, Luscombe VB, Stauffer SR, Lindsley CW, Conn PJ, Meiler J Abstract As part of the G-protein coupled receptor (GPCR) family, ... read more

Related ArticlesReduced nucleus accumbens enkephalins underlie vulnerability to social defeat stress. Neuropsychopharmacology. 2019 May 27;: Authors: Nam H, Chandra R, Francis TC, Dias C, Cheer JF, Lobo MK Abstract Enkephalins, endogenous ligands for delta opioid receptors (DORs), are highly enriched in the nucleus accumbens (NAc). They are implicated in depression ... read more

Related ArticlesMast Cells in Neurodegenerative Disease. Front Cell Neurosci. 2019;13:171 Authors: Jones MK, Nair A, Gupta M Abstract Neurodegenerative diseases affect millions of people worldwide, yet there are currently no effective treatments. Because risk of neurodegenerative disease substantially increases with age, greater life expectancy with a concomitant aging population means ... read more

Related ArticlesLongitudinal characterization of cognitive and motor deficits in an excitotoxic lesion model of striatal dysfunction in non-human primates. Neurobiol Dis. 2019 May 24;:104484 Authors: Lavisse S, Williams S, Lecourtois S, van Camp N, Guillermier M, Gipchtein P, Jan C, Goutal S, Eymin L, Valette J, Delzescaux T, Perrier AL, ... read more

Related ArticlesQuantitation of Neurotoxic Metabolites of the Kynurenine Pathway by Laser Desorption Ionization Mass Spectrometry (LDI-MS). Methods Mol Biol. 2019;1996:113-129 Authors: Lahiri P, Dhaware D, Singh A, Panchagnula V, Ghosh D Abstract The metabolites of the mammalian kynurenine (KYN) pathway are generated from a branch of tryptophan metabolic pathway. The ... read more

Related ArticlesMethods to Study Trinucleotide Repeat Instability Induced by DNA Damage and Repair. Methods Mol Biol. 2019;1999:87-101 Authors: Lai Y, Lei R, Ren Y, Liu Y Abstract Trinucleotide repeat (TNR) instability (expansion and deletion) is associated with more than 42 human neurodegenerative diseases and cancer and mediated by DNA replication, ... read more

Related ArticlesTALEN-Induced Double-Strand Break Repair of CTG Trinucleotide Repeats. Cell Rep. 2018 02 20;22(8):2146-2159 Authors: Mosbach V, Poggi L, Viterbo D, Charpentier M, Richard GF Abstract Trinucleotide repeat expansions involving CTG/CAG triplets are responsible for several neurodegenerative disorders, including myotonic dystrophy and Huntington's disease. Because expansions trigger the disease, contracting ... read more

Related ArticlesSpinocerebellar ataxia 48 presenting with ataxia associated with cognitive, psychiatric, and extrapyramidal features: A report of two Italian families. Parkinsonism Relat Disord. 2019 May 14;: Authors: De Michele G, Lieto M, Galatolo D, Salvatore E, Cocozza S, Barghigiani M, Tessa A, Baldacci J, Pappatà S, Filla A, De Michele ... read more

Related ArticlesExtrastriatal degeneration correlates with deficits in the motor domain subscales of the UHDRS. J Neurol Sci. 2018 02 15;385:22-29 Authors: Galvez V, Ramírez-García G, Hernandez-Castillo CR, Bayliss L, Díaz R, Lopez-Titla MM, Campos-Romo A, Fernandez-Ruiz J Abstract INTRODUCTION: Striatal degeneration has significant behavioral effects in patients with Huntington's ... read more

Related ArticlesA promising orphan target to treat HD. Mov Disord. 2018 11;33(11):1674 Authors: Ratna N PMID: 30485911 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesThe effects of cognitive behavioral and mindfulness-based therapies on psychological distress in patients with multiple sclerosis, Parkinson's disease and Huntington's disease: Two meta-analyses. J Psychosom Res. 2019 Jul;122:43-51 Authors: Ghielen I, Rutten S, Boeschoten RE, Houniet-de Gier M, van Wegen EEH, van den Heuvel OA, Cuijpers P Abstract OBJECTIVE: ... read more

Metformin reverses the schizophrenia-like behaviors induced by MK-801 in rats. Brain Res. 2019 May 20;: Authors: Wang X, Luo C, Mao XY, Li X, Yin JY, Zhang W, Zhou HH, Liu ZQ Abstract Schizophrenia is known to be a complex and disabling psychiatric disorder. Dopamine receptor antagonists have a significant ... read more

STAT1 drives M1 microglia activation and neuroinflammation under hypoxia. Arch Biochem Biophys. 2019 May 20;: Authors: Butturini E, Boriero D, Carcereri de Prati A, Mariotto S Abstract Microglia are resident immune cells that act as the first active defence in the central nervous system. These cells constantly monitor the tissue ... read more

Related ArticlesAntisense oligonucleotides: A primer. Neurol Genet. 2019 Apr;5(2):e323 Authors: Scoles DR, Minikel EV, Pulst SM Abstract There are few disease-modifying therapeutics for neurodegenerative diseases, but successes on the development of antisense oligonucleotide (ASO) therapeutics for spinal muscular atrophy and Duchenne muscular dystrophy predict a robust future for ASOs in ... read more

Related ArticlesDetermining the Roles of Inositol Trisphosphate Receptors in Neurodegeneration: Interdisciplinary Perspectives on a Complex Topic. Mol Neurobiol. 2017 11;54(9):6870-6884 Authors: Takada SH, Ikebara JM, de Sousa E, Cardoso DS, Resende RR, Ulrich H, Rückl M, Rüdiger S, Kihara AH Abstract It is well known that calcium (Ca2+) is ... read more

Related ArticlesPatients with Huntington's disease pioneered human stereotactic neurosurgery 70 years ago. Brain. 2017 09 01;140(9):2516-2519 Authors: Hariz M, Tabrizi S PMID: 29050388 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesPope Francis champions Huntington's disease. Brain. 2017 09 01;140(9):e58 Authors: Spinney L PMID: 29050385 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesSubcortical neurodegeneration in chorea: Similarities and differences between chorea-acanthocytosis and Huntington's disease. Parkinsonism Relat Disord. 2018 04;49:54-59 Authors: Liu J, Heinsen H, Grinberg LT, Alho E, Amaro E, Pasqualucci CA, Rüb U, den Dunnen W, Arzberger T, Schmitz C, Kiessling M, Bader B, Danek A Abstract INTRODUCTION: Chorea-acanthocytosis ... read more

Related ArticlesThe contribution of gender differences in motor, behavioral and cognitive features to functional capacity, independence and quality of life in patients with Huntington's disease. Parkinsonism Relat Disord. 2018 04;49:42-47 Authors: Zielonka D, Ren M, De Michele G, Roos RAC, Squitieri F, Bentivoglio AR, Marcinkowski JT, Landwehrmeyer GB Abstract ... read more

Related ArticlesGenetic Counseling in Huntington's Disease: Potential New Challenges on Horizon? Front Neurol. 2019;10:453 Authors: Migliore S, Jankovic J, Squitieri F Abstract Huntington's disease (HD) is a rare, hereditary, neurodegenerative and dominantly transmitted disorder affecting about 10 out of 100,000 people in Western Countries. The genetic cause is a CAG ... read more

Psychosocial Impact of Dysarthria: The Patient-Reported Outcome as Part of the Clinical Management. Neurodegener Dis. 2019 May 21;:1-10 Authors: Atkinson-Clement C, Letanneux A, Baille G, Cuartero MC, Véron-Delor L, Robieux C, Berthelot M, Robert D, Azulay JP, Defebvre L, Ferreira J, Eusebio A, Moreau C, Pinto S Abstract BACKGROUND: Dysarthria ... read more

Related ArticlesMolecular mechanisms of heterogeneous oligomerization of huntingtin proteins. Sci Rep. 2019 May 20;9(1):7615 Authors: Bonfanti S, Lionetti MC, Fumagalli MR, Chirasani VR, Tiana G, Dokholyan NV, Zapperi S, La Porta CAM Abstract There is still no successful strategy to treat Huntington's disease, an inherited autosomal disorder associated with the ... read more

Related ArticlesEndogenous mouse huntingtin is highly abundant in cranial nerve nuclei, co-aggregates to Abeta plaques and is induced in reactive astrocytes in a transgenic mouse model of Alzheimer's disease. Acta Neuropathol Commun. 2019 May 20;7(1):79 Authors: Hartlage-Rübsamen M, Ratz V, Zeitschel U, Finzel L, Machner L, Köppen J, Schulze A, ... read more

Related ArticlesLength of Uninterrupted CAG, Independent of Polyglutamine Size, Results in Increased Somatic Instability, Hastening Onset of Huntington Disease. Am J Hum Genet. 2019 May 08;: Authors: Wright GEB, Collins JA, Kay C, McDonald C, Dolzhenko E, Xia Q, Bečanović K, Drögemöller BI, Semaka A, Nguyen CM, Trost B, Richards ... read more

Rare homozygosity in amyotrophic lateral sclerosis suggests the contribution of recessive variants to disease genetics. J Neurol Sci. 2019 May 08;402:62-68 Authors: Goldstein O, Kedmi M, Gana-Weisz M, Twito S, Nefussy B, Vainer B, Fainmesser Y, Abraham A, Nayshool O, Orr-Urtreger A, Drory VE Abstract OBJECTIVE: to determine the occurrence ... read more

Pridopidine protects neurons from mutant-huntingtin toxicity via the sigma-1 receptor. Neurobiol Dis. 2019 May 17;: Authors: Eddings CR, Arbez N, Akimov S, Geva M, Hayden MR, Ross CA Abstract Huntington's disease (HD) is a neurodegenerative disease caused by a CAG repeat expansion in the Huntingtin gene (HTT), translated into a ... read more

Related ArticlesSelf-assembly of Mutant Huntingtin Exon-1 Fragments into Large Complex Fibrillar Structures Involves Nucleated Branching. J Mol Biol. 2018 06 08;430(12):1725-1744 Authors: Wagner AS, Politi AZ, Ast A, Bravo-Rodriguez K, Baum K, Buntru A, Strempel NU, Brusendorf L, Hänig C, Boeddrich A, Plassmann S, Klockmeier K, Ramirez-Anguita JM, ... read more

EGF Treatment Improves Motor Behavior and Cortical GABAergic Function in the R6/2 Mouse Model of Huntington's Disease. Mol Neurobiol. 2019 May 19;: Authors: Marottoli FM, Priego M, Flores-Barrera E, Pisharody R, Zaldua S, Fan KD, Ekkurthi GK, Brady ST, Morfini GA, Tseng KY, Tai LM Abstract Recent evidence indicates that ... read more

Improved functional capacity in Huntington disease after Nordic walking training: A case report. Neurologia. 2019 May 15;: Authors: Martínez-Lemos RI, Domínguez-Pérez C, Seijo-Martínez M, Ayán-Pérez C PMID: 31103310 [PubMed - as supplied by publisher] ... read more

Clinical phenotype in carriers of intermediate alleles in the huntingtin gene. J Neurol Sci. 2019 May 13;402:57-61 Authors: Savitt D, Jankovic J Abstract OBJECTIVE: To describe the phenotype of individuals with intermediate allele (IA) CAG repeat length in the huntingtin (HTT) gene evaluated at the Parkinson's Disease Center and Movement ... read more

Related ArticlesPhysical ACtivity and Exercise Outcomes in Huntington Disease (PACE-HD): Protocol for a 12-Month Trial Within Cohort Evaluation of a Physical Activity Intervention in People With Huntington Disease. Phys Ther. 2019 May 17;: Authors: Drew CJG, Quinn L, Hamana K, Williams-Thomas R, Marsh L, Dimitropoulou P, Playle R, Griffin BA, ... read more

Related ArticlesDrp1 phosphorylation by MAPK1 causes mitochondrial dysfunction in cell culture model of Huntington's disease. Biochem Biophys Res Commun. 2018 02 05;496(2):706-711 Authors: Roe AJ, Qi X Abstract Mitochondrial dysfunction is a major cytopathology in Huntington's disease (HD), a fatal and inherited neurodegenerative disease. However, the molecular ... read more

Related ArticlesTargeted Oligonucleotides for Treating Neurodegenerative Tandem Repeat Diseases. Neurotherapeutics. 2019 May 16;: Authors: Zain R, Smith CIE Abstract Nucleotide repeat disorders encompass more than 30 diseases, most of which show dominant inheritance, such as Huntington's disease, spinocerebellar ataxias, and myotonic dystrophies. Yet others, including Friedreich's ataxia, are recessively inherited. ... read more

Alterations in Synaptic Function and Plasticity in Huntington Disease. J Neurochem. 2019 May 16;: Authors: Smith-Dijak AI, Sepers MD, Raymond LA Abstract Huntington disease (HD) is an inherited neurodegenerative disorder caused by an expansion of the CAG repeat region in the first exon of the huntingtin (Htt) gene. Neurodegeneration, which ... read more

Related ArticlesSingle-cell mass cytometry reveals distinct populations of brain myeloid cells in mouse neuroinflammation and neurodegeneration models. Nat Neurosci. 2018 04;21(4):541-551 Authors: Ajami B, Samusik N, Wieghofer P, Ho PP, Crotti A, Bjornson Z, Prinz M, Fantl WJ, Nolan GP, Steinman L Abstract Neuroinflammation and neurodegeneration may represent two ... read more

Related ArticlesThe choice not to undergo genetic testing for Huntington disease: Results from the PHAROS study. Clin Genet. 2019 May 15;: Authors: Anderson KE, Eberly S, Marder KS, Oakes D, Kayson E, Young A, Shoulson I, PHAROS Investigators Abstract Rates of genetic testing in Huntington disease (HD) are lower than ... read more

Related ArticlesAge of onset in Huntington's disease is influenced by CAG repeat variations in other polyglutamine disease-associated genes. Brain. 2017 07 01;140(7):e42 Authors: Stuitje G, van Belzen MJ, Gardiner SL, van Roon-Mom WMC, Boogaard MW, REGISTRY Investigators of the European Huntington Disease Network, Tabrizi SJ, Roos RAC, Aziz NA ... read more

Related ArticlesPredictive testing and clinical trials in Huntington's disease: An ethical analysis. Mov Disord. 2018 02;33(2):243-247 Authors: Sampaio C, Levey J, Klitzman R PMID: 29205501 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesRating scales for cognition in Huntington's disease: Critique and recommendations. Mov Disord. 2018 02;33(2):187-195 Authors: Mestre TA, Bachoud-Lévi AC, Marinus J, Stout JC, Paulsen JS, Como P, Duff K, Sampaio C, Goetz CG, Cubo E, Stebbins GT, Martinez-Martin P, Members of the MDS Committee on Rating Scales Development ... read more

Related ArticlesHTT haplogroups in Finnish patients with Huntington disease. Neurol Genet. 2019 Jun;5(3):e334 Authors: Ylönen S, Sipilä JOT, Hietala M, Majamaa K Abstract Objective: To study genetic causes of the low frequency of Huntington disease (HD) in the Finnish population, we determined HTT haplogroups in the population and patients with ... read more