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Related ArticlesRare case of chorea-hyperglycaemia-basal ganglia (C-H-BG) syndrome. BMJ Case Rep. 2018 Apr 17;2018: Authors: Ahmad S, Mohan Babu P, Shenbagaraj L, George L Abstract An 83-year-old woman presented with acute-onset haemichorea and haemiballism particularly affecting the left side of the body. She was known to have type 2 diabetes, ... read more

A whole brain longitudinal study in the YAC128 mouse model of Huntington's disease shows distinct trajectories of neurochemical, structural connectivity and volumetric changes. Hum Mol Genet. 2018 Apr 16;: Authors: Petrella LI, Castelhano JM, Ribeiro M, Sereno JV, Gonçalves SI, Laço MN, Hayden MR, Rego AC, Castelo-Branco M Abstract Huntington's ... read more

Related ArticlesDoes Vitamin C Influence Neurodegenerative Diseases and Psychiatric Disorders? Nutrients. 2017 Jun 27;9(7): Authors: Kocot J, Luchowska-Kocot D, Kiełczykowska M, Musik I, Kurzepa J Abstract Vitamin C (Vit C) is considered to be a vital antioxidant molecule in the brain. Intracellular Vit C helps maintain integrity and function ... read more

Deutetrabenazine (Austedo) for Huntington's chorea and tardive dyskinesia. Med Lett Drugs Ther. 2018 Apr 23;60(1545):65-68 Authors: PMID: 29667946 [PubMed - in process] ... read more

Related ArticlesAlterations of striatal indirect pathway neurons precede motor deficits in two mouse models of Huntington's disease. Neurobiol Dis. 2017 Sep;105:117-131 Authors: Sebastianutto I, Cenci MA, Fieblinger T Abstract Striatal neurons forming the indirect pathway (iSPNs) are particularly vulnerable in Huntington's disease (HD). In this study we set out ... read more

Related ArticlesRegulation of motor proteins, axonal transport deficits and adult-onset neurodegenerative diseases. Neurobiol Dis. 2017 Sep;105:273-282 Authors: Brady ST, Morfini GA Abstract Neurons affected in a wide variety of unrelated adult-onset neurodegenerative diseases (AONDs) typically exhibit a "dying back" pattern of degeneration, which is characterized by early deficits in ... read more

Related ArticlesTreadmill exercise delays the onset of non-motor behaviors and striatal pathology in the CAG140 knock-in mouse model of Huntington's disease. Neurobiol Dis. 2017 Sep;105:15-32 Authors: Stefanko DP, Shah VD, Yamasaki WK, Petzinger GM, Jakowec MW Abstract Depression, cognitive impairments, and other neuropsychiatric disturbances are common during the prodromal ... read more

Related ArticlesDriving with a neurodegenerative disorder: an overview of the current literature. J Neurol. 2017 Aug;264(8):1678-1696 Authors: Jacobs M, Hart EP, Roos RAC Abstract Driving is important for employment, social activities, and for the feeling of independence. The decision to cease driving affects the quality of life and ... read more

Related ArticlesDisruption of GABA(A)-mediated intracortical inhibition in patients with chorea-acanthocytosis. Neurosci Lett. 2017 Jul 27;654:107-110 Authors: Dubbioso R, Esposito M, Peluso S, Iodice R, De Michele G, Santoro L, Manganelli F Abstract Chorea-acanthocytosis (Ch-Ac) is an autosomal recessive neurodegenerative disorder characterized by adult-onset chorea, acanthocytes in the peripheral blood, ... read more

Longitudinal Anthropometric Assessment of Rhesus Macaque (Macaca mulatta) Model of Huntington Disease. Comp Med. 2018 Apr 02;68(2):163-167 Authors: Hunter CE, Pongos AL, Chi TY, Payne C, Stroud FC, Chan AWS Abstract The neurodegeneration associated with Huntington disease (HD) leads to the onset of motor and cognitive impairment and their advancement ... read more

Related ArticlesGenetically modified rodent models of SCA17. J Neurosci Res. 2017 Aug;95(8):1540-1547 Authors: Cui Y, Yang S, Li XJ, Li S Abstract Spinocerebellar ataxia type 17 (SCA17) is a type of autosomal dominant cerebellar ataxia (ADCA) characterized by variable manifestations, including cerebellar ataxia, dementia, and psychiatric symptoms. Since ... read more

Related ArticlesSeventy years of pallidotomy for movement disorders. Mov Disord. 2017 Jul;32(7):972-982 Authors: Cif L, Hariz M Abstract The year 2017 marks the 70th anniversary of the birth of human stereotactic neurosurgery. The first procedure was a pallidotomy for Huntington's disease. However, it was for Parkinson's disease that pallidotomy ... read more

Related ArticlesObjective assessment of gait and posture in premanifest and manifest Huntington disease - A multi-center study. Gait Posture. 2018 Apr 07;62:451-457 Authors: Beckmann H, Bohlen S, Saft C, Hoffmann R, Gerss J, Muratori L, Ringelstein EB, Landwehrmeyer GB, Reilmann R Abstract BACKGROUND: Deficits in posture and gait are known ... read more

Related ArticlesPatient-reported outcomes in Huntington's disease: Quality of life in neurological disorders (Neuro-QoL) and Huntington's disease health-related quality of life (HDQLIFE) physical function measures. Mov Disord. 2017 Jul;32(7):1096-1102 Authors: Carlozzi NE, Ready RE, Frank S, Cella D, Hahn EA, Goodnight SM, Schilling SG, Boileau NR, Dayalu P Abstract BACKGROUND: ... read more

Related ArticlesChorea in the older adult: a full blooded answer. J R Coll Physicians Edinb. 2016 Dec;46(4):244-246 Authors: Degnan AJ, Capek E, Bowman A Abstract Chorea is a severe, distressing, movement disorder characterised by excessive, purposeless movements of the limbs, head and orofacial muscles in a generalised and irregularly-timed fashion. ... read more

Related ArticlesSIRT2 Inhibition Confers Neuroprotection by Downregulation of FOXO3a and MAPK Signaling Pathways in Ischemic Stroke. Mol Neurobiol. 2018 Apr 14;: Authors: She DT, Wong LJ, Baik SH, Arumugam TV Abstract Sirtuin 2 (SIRT2) is a family member of nicotinamide adenine dinucleotide (NAD+)-dependent deacetylases which appears to have detrimental roles ... read more

Related ArticlesTranscriptomic Biomarkers for Huntington's Disease: Are Gene Expression Signatures in Whole Blood Reliable Biomarkers? OMICS. 2018 Apr;22(4):283-294 Authors: Zadel M, Maver A, Kovanda A, Peterlin B Abstract Huntington's disease (HD) is a severe neurodegenerative disorder manifesting as progressive impairment of motor function, cognitive decline, psychiatric symptoms, and immunological and ... read more

Related ArticlesNeuropeptide Y mitigates ER stress-induced neuronal cell death by activating the PI3K-XBP1 pathway. Eur J Cell Biol. 2018 Apr 06;: Authors: Lee DY, Hong SH, Kim B, Lee DS, Yu K, Lee KS Abstract The unfolded protein response (UPR) is an evolutionarily conserved adaptive reaction that increases cell survival ... read more

Related ArticlesGenetics Modulate Gray Matter Variation Beyond Disease Burden in Prodromal Huntington's Disease. Front Neurol. 2018;9:190 Authors: Liu J, Ciarochi J, Calhoun VD, Paulsen JS, Bockholt HJ, Johnson HJ, Long JD, Lin D, Espinoza FA, Misiura MB, Caprihan A, Turner JA, PREDICT-HD Investigators and Coordinators of the Huntington Study Group ... read more

Related ArticlesFUS/TLS acts as an aggregation-dependent modifier of polyglutamine disease model mice. Sci Rep. 2016 10 14;6:35236 Authors: Kino Y, Washizu C, Kurosawa M, Yamada M, Doi H, Takumi T, Adachi H, Katsuno M, Sobue G, Hicks GG, Hattori N, Shimogori T, Nukina N Abstract FUS/TLS is an ... read more

Environment-dependent striatal gene expression in the BACHD rat model for Huntington disease. Sci Rep. 2018 Apr 11;8(1):5803 Authors: Novati A, Hentrich T, Wassouf Z, Weber JJ, Yu-Taeger L, Déglon N, Nguyen HP, Schulze-Hentrich JM Abstract Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a mutation in the ... read more

Publisher Correction: Alteration in Fluidity of Cell Plasma Membrane in Huntington Disease Revealed by Spectral Phasor Analysis. Sci Rep. 2018 Apr 11;8(1):6060 Authors: Sameni S, Malacrida L, Tan Z, Digman MA Abstract A correction to this article has been published and is linked from the HTML and PDF versions of ... read more

The Effect of Tissue Preparation and Donor Age on Striatal Graft Morphology in the Mouse. Cell Transplant. 2018 Feb;27(2):230-244 Authors: Harrison DJ, Roberton VH, Vinh NN, Brooks SP, Dunnett SB, Rosser AE Abstract Huntington's disease (HD) is a progressive neurodegenerative disease in which striatal medium spiny neurons (MSNs) are lost. ... read more

Glial alterations in human prion diseases: A correlative study of astroglia, reactive microglia, protein deposition, and neuropathological lesions. Medicine (Baltimore). 2018 Apr;97(15):e0320 Authors: Monzón M, Hernández RS, Garcés M, Sarasa R, Badiola JJ Abstract BACKGROUND: Neuroinflammation has recently been proposed to be a major component of neurodegenerative diseases. The aim ... read more

Related ArticlesMini-symposium: Huntington's disease - introduction. Brain Pathol. 2016 11;26(6):724-725 Authors: Ferrer I PMID: 27529604 [PubMed - indexed for MEDLINE] ... read more

Related ArticlesDeficits in temporal processing correlate with clinical progression in Huntington's disease. Acta Neurol Scand. 2017 Oct;136(4):322-329 Authors: Agostino PV, Gatto EM, Cesarini M, Etcheverry JL, Sanguinetti A, Golombek DA Abstract OBJECTIVES: Precise temporal performance is crucial for several complex tasks. Time estimation in the second-to-minutes range-known as interval ... read more

Related ArticlesLongitudinal modeling of appearance and shape and its potential for clinical use. Med Image Anal. 2016 Oct;33:114-121 Authors: Gerig G, Fishbaugh J, Sadeghi N Abstract Clinical assessment routinely uses terms such as development, growth trajectory, degeneration, disease progression, recovery or prediction. This terminology inherently carries the aspect ... read more

Related ArticlesPallidal Deep Brain Stimulation in Patients With Chorea-Acanthocytosis. Neuromodulation. 2018 Apr 10;: Authors: Liu Z, Liu Y, Wan X, Yang Y, Wang L, Dou W, Guo J, Wang Y, Guo Y, Ma W, Wang R Abstract INTRODUCTION: Chorea-acanthocytosis (ChAc) is an autosomal recessive hereditary disorder caused by the mutation ... read more

Related ArticlesAutophagy is increased following either pharmacological or genetic silencing of mGluR5 signaling in Alzheimer's disease mouse models. Mol Brain. 2018 Apr 10;11(1):19 Authors: Abd-Elrahman KS, Hamilton A, Vasefi M, Ferguson SSG Abstract Alzheimer's disease (AD) is characterized by neurotoxicity mediated by the accumulation of beta amyloid (Aβ) oligomers, causing ... read more

Related ArticlesLongitudinal Anthropometric Assessment of Rhesus Macaque (Macaca mulatta) Model of Huntington Disease. Comp Med. 2019 Mar 01;68(2):163-167 Authors: Hunter CE, Pongos AL, Chi TY, Payne C, Stroud FC, Chan AWS Abstract The neurodegeneration associated with Huntington disease (HD) leads to the onset of motor and cognitive impairment and their ... read more

Related ArticlesSigma-1 (σ1) Receptor in Memory and Neurodegenerative Diseases. Handb Exp Pharmacol. 2017;244:81-108 Authors: Maurice T, Goguadze N Abstract The sigma-1 (σ1) receptor has been associated with regulation of intracellular Ca2+ homeostasis, several cellular signaling pathways, and inter-organelle communication, in part through its chaperone activity. In vivo, agonists of ... read more

Brain pericyte activation occurs early in Huntington's disease. Exp Neurol. 2018 Apr 06;: Authors: Padel T, Roth M, Gaceb A, Li JY, Björkqvist M, Paul G Abstract Microvascular changes have recently been described for several neurodegenerative disorders, including Huntington's disease (HD). HD is characterized by a progressive neuronal cell loss ... read more

Importance of the subcellular location of protein deposits in neurodegenerative diseases. Curr Opin Neurobiol. 2018 Apr 06;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 features: ... read more

XJB-5-131-mediated improvement in physiology and behaviour of the R6/2 mouse model of Huntington's disease is age- and sex- dependent. PLoS One. 2018;13(4):e0194580 Authors: Polyzos AA, Wood NI, Williams P, Wipf P, Morton AJ, McMurray CT Abstract We have reported that the radical scavenger XJB-5-131 attenuates or reverses progression of the ... read more

Does N-terminal huntingtin function as a 'holdase' for inhibiting cellular protein aggregation? FEBS J. 2018 Apr 06;: 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 in ... read more

Related ArticleshPSC-Derived Striatal Cells Generated Using a Scalable 3D Hydrogel Promote Recovery in a Huntington Disease Mouse Model. Stem Cell Reports. 2018 Apr 03;: Authors: Adil MM, Gaj T, Rao AT, Kulkarni RU, Fuentes CM, Ramadoss GN, Ekman FK, Miller EW, Schaffer DV Abstract Huntington disease (HD) is an inherited, ... read more

Tadpole-like conformations of huntingtin exon 1 are characterized by conformational heterogeneity that persists regardless of polyglutamine length. J Mol Biol. 2018 Apr 05;: Authors: Newcombe EA, Ruff KM, Sethi A, Ormsby AR, Ramdzan YM, Fox A, Purcell AW, Gooley PR, Pappu RV, Hatters DM Abstract Soluble huntingtin exon 1 (Httex1) ... read more

Related ArticlesWhat to Do with a Second Chance in Life? Long-Term Experiences of Non-carriers of Huntington's Disease. J Genet Couns. 2018 Apr 07;: Authors: Winnberg E, Winnberg U, Pohlkamp L, Hagberg A Abstract Little is known about how people's lives are influenced when going from a 50% risk status of ... read more

Brain mitochondrial iron accumulates in Huntington's disease, mediates mitochondrial dysfunction, and can be removed pharmacologically. Free Radic Biol Med. 2018 Apr 03;: Authors: Agrawal S, Fox J, Thyagarajan B, Fox J Abstract Mitochondrial bioenergetic dysfunction is involved in neurodegeneration in Huntington's disease (HD). Iron is critical for normal mitochondrial bioenergetics ... read more

Receptor for Advanced Glycation End Products (RAGE) is expressed predominantly in Medium Spiny Neurons of tgHD rat striatum. Neuroscience. 2018 Apr 03;: Authors: Shi D, Chang JWH, Choi J, Connor B, O'Carroll S, Nicholson LFB, Kim JH Abstract Receptor for advanced glycation end products (RAGE) is a multi-ligand receptor involved ... read more

Related ArticlesOn the Role of Store-Operated Calcium Entry in Acute and Chronic Neurodegenerative Diseases. Front Mol Neurosci. 2018;11:87 Authors: Secondo A, Bagetta G, Amantea D Abstract In both excitable and non-excitable cells, calcium (Ca2+) signals are maintained by a highly integrated process involving store-operated Ca2+ entry (SOCE), namely the opening ... read more

Related ArticlesInsights into the mechanisms of copper dyshomeostasis in amyotrophic lateral sclerosis. Expert Rev Mol Med. 2017 Jun 09;19:e7 Authors: Gil-Bea FJ, Aldanondo G, Lasa-Fernández H, López de Munain A, Vallejo-Illarramendi A Abstract Amyotrophic lateral sclerosis (ALS) is a severe neuromuscular disease characterised by a progressive loss of motor neurons ... read more

Related ArticlesChinese Medicine: A Hope for Neurodegenerative Diseases? J Alzheimers Dis. 2017;60(s1):S151-S160 Authors: Law BYK, Wu AG, Wang MJ, Zhu YZ Abstract With the increase in the proportion of aged population due to the rapid increase of life expectancy, the worldwide prevalence rate of multiple neurodegenerative diseases including Alzheimer's ... read more

ROCK inhibition in models of neurodegeneration and its potential for clinical translation. Pharmacol Ther. 2018 Apr 02;: Authors: Koch JC, Tatenhorst L, Roser AE, Saal KA, Tönges L, Lingor P Abstract Neurodegenerative disorders like Parkinson's disease, Alzheimer's disease, or amyotrophic lateral sclerosis are affecting a rapidly increasing population worldwide. While ... read more

Visual scanning of the eye region of human faces predicts emotion recognition performance in Huntington's disease. Neuropsychology. 2018 Mar;32(3):356-365 Authors: Kordsachia CC, Labuschagne I, Stout JC Abstract OBJECTIVE: Previous research has consistently shown that the ability to recognize emotions from facial expressions is impaired in Huntington's disease (HD). The aim ... read more

Proteostasis in Huntington's disease: disease mechanisms and therapeutic opportunities. Acta Pharmacol Sin. 2018 Apr 05;: Authors: Harding RJ, Tong YF Abstract Many neurodegenerative diseases are characterized by impairment of protein quality control mechanisms in neuronal cells. Ineffective clearance of misfolded proteins by the proteasome, autophagy pathways and exocytosis leads to ... read more

An Optical Neuron-Astrocyte Proximity Assay at Synaptic Distance Scales. Neuron. 2018 Apr 04;98(1):49-66.e9 Authors: Octeau JC, Chai H, Jiang R, Bonanno SL, Martin KC, Khakh BS Abstract Astrocytes are complex bushy cells that serve important functions through close contacts between their processes and synapses. However, the spatial interactions and dynamics of ... read more

Dose-Dependent Lowering of Mutant Huntingtin Using Antisense Oligonucleotides in Huntington Disease Patients. Nucleic Acid Ther. 2018 Apr;28(2):59-62 Authors: van Roon-Mom WMC, Roos RAC, de Bot ST Abstract On December 11 of 2017, Ionis Pharmaceuticals published a press release announcing dose-dependent reductions of mutant huntingtin protein in their HTTRx Phase 1/2a ... read more

Related ArticlesEffect of Kynurenic Acid on Pupae Viability of Drosophila melanogaster cinnabar and cardinal Eye Color Mutants with Altered Tryptophan-Kynurenine Metabolism. Neurotox Res. 2018 Apr 04;: Authors: Navrotskaya V, Wnorowski A, Turski W, Oxenkrug G Abstract Kynurenic acid (KYNA) is one of the metabolites of evolutionary conserved tryptophan (Trp)/kynurenine (Kyn) ... read more

Related ArticlesA Study of Triplet-Primed PCR for Identification of CAG Repeat Expansion in the HTT Gene in a Cohort of 503 Indian Cases with Huntington's Disease Symptoms. Mol Diagn Ther. 2018 Apr 04;: Authors: Chheda P, Chanekar M, Salunkhe Y, Dama T, Pais A, Pande S, Bendre R, Shah N ... read more