11,652 results match your criteria Huntington Disease Dementia


Blood-brain barrier breakdown is an early biomarker of human cognitive dysfunction.

Nat Med 2019 Feb 14;25(2):270-276. Epub 2019 Jan 14.

Department of Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.

Vascular contributions to cognitive impairment are increasingly recognized as shown by neuropathological, neuroimaging, and cerebrospinal fluid biomarker studies. Moreover, small vessel disease of the brain has been estimated to contribute to approximately 50% of all dementias worldwide, including those caused by Alzheimer's disease (AD). Vascular changes in AD have been typically attributed to the vasoactive and/or vasculotoxic effects of amyloid-β (Aβ), and more recently tau. Read More

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http://www.nature.com/articles/s41591-018-0297-y
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http://dx.doi.org/10.1038/s41591-018-0297-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367058PMC
February 2019
6 Reads
27.363 Impact Factor

Vascular dysfunction-The disregarded partner of Alzheimer's disease.

Alzheimers Dement 2019 Jan;15(1):158-167

Department of Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA; Alzheimer's Disease Research Center, Keck School of Medicine at the University of Southern California, Los Angeles, CA, USA. Electronic address:

Increasing evidence recognizes Alzheimer's disease (AD) as a multifactorial and heterogeneous disease with multiple contributors to its pathophysiology, including vascular dysfunction. The recently updated AD Research Framework put forth by the National Institute on Aging-Alzheimer's Association describes a biomarker-based pathologic definition of AD focused on amyloid, tau, and neuronal injury. In response to this article, here we first discussed evidence that vascular dysfunction is an important early event in AD pathophysiology. Read More

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http://dx.doi.org/10.1016/j.jalz.2018.07.222DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6338083PMC
January 2019
25 Reads
12.407 Impact Factor

Data-Driven Based Approach to Aid Parkinson's Disease Diagnosis.

Sensors (Basel) 2019 Jan 10;19(2). Epub 2019 Jan 10.

Laboratory of Images, Signals and Intelligent Systems (LISSI), University of Paris-Est Créteil (UPEC), 122 rue Paul Armangot, 94400 Vitry-Sur-Seine, France.

This article presents a machine learning methodology for diagnosing Parkinson's disease (PD) based on the use of vertical Ground Reaction Forces (vGRFs) data collected from the gait cycle. A classification engine assigns subjects to healthy or Parkinsonian classes. The diagnosis process involves four steps: data pre-processing, feature extraction and selection, data classification and performance evaluation. Read More

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http://dx.doi.org/10.3390/s19020242DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6359125PMC
January 2019
6 Reads

A smartphone application for automated decision support in cognitive task based evaluation of central nervous system motor disorders.

IEEE J Biomed Health Inform 2019 Jan 9. Epub 2019 Jan 9.

Background And Objective: New technology enables constant boost to the powers of mobile devices, which in the previous years have transformed from simple mobile phones to smart phones. Computational powers of these electronics enable actions that previously were possible only for computers. By the use of special applications we may benefit from sensors and multimedia capabilities of operating systems. Read More

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https://ieeexplore.ieee.org/document/8606105/
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http://dx.doi.org/10.1109/JBHI.2019.2891729DOI Listing
January 2019
7 Reads

An active cognitive lifestyle as a potential neuroprotective factor in Huntington's disease.

Neuropsychologia 2019 01 26;122:116-124. Epub 2018 Oct 26.

Cognition and Brain Plasticity Unit, Neuroscience Program, IDIBELL (Institut d'Investigació Biomèdica de Bellvitge), L'Hospitalet de Llobregat, Barcelona, Spain. Electronic address:

A cognitive stimulating lifestyle has been observed to confer cognitive benefits in multiple neurodegenerative diseases. However, the underlying neurobiological basis of this phenomenon remains unclear. Huntington's disease can provide a suitable model to study the effects and neural mechanisms of cognitive engagement in neurodegeneration. Read More

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http://dx.doi.org/10.1016/j.neuropsychologia.2018.10.017DOI Listing
January 2019
9 Reads

Molecular Pathogenesis in Huntington's Disease.

Biochemistry (Mosc) 2018 Sep;83(9):1030-1039

Institute of Cytology, Russian Academy of Sciences, St. Petersburg, 194064, Russia.

Huntington's disease (HD) is a severe autosomal dominant neurodegenerative disorder characterized by a combination of motor, cognitive, and psychiatric symptoms, atrophy of the basal ganglia and the cerebral cortex, and inevitably progressive course resulting in death 5-20 years after manifestation of its symptoms. HD is caused by expansion of CAG repeats in the HTT gene, which leads to pathological elongation of the polyglutamine tract within the respective protein - huntingtin. In this review, we present a modern view on molecular biology of HD as a representative of the group of polyglutamine diseases, with an emphasis on conformational changes of mutant huntingtin, disturbances in its cellular processing, and proteolytic stress in degenerating neurons. Read More

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http://dx.doi.org/10.1134/S0006297918090043DOI Listing
September 2018
11 Reads

A cross-brain regions study of ANK1 DNA methylation in different neurodegenerative diseases.

Neurobiol Aging 2019 Feb 13;74:70-76. Epub 2018 Oct 13.

University of Exeter Medical School, University of Bristol, Exeter, UK. Electronic address:

Recent epigenome-wide association studies in Alzheimer's disease have highlighted consistent robust neuropathology-associated DNA hypermethylation of the ankyrin 1 (ANK1) gene in the cortex. The extent to which altered ANK1 DNA methylation is also associated with other neurodegenerative diseases is not currently known. In the present study, we used bisulfite pyrosequencing to quantify DNA methylation across 8 CpG sites within a 118 bp region of the ANK1 gene across multiple brain regions in Alzheimer's disease, Vascular dementia, Dementia with Lewy bodies, Huntington's disease, and Parkinson's disease. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2018.09.024DOI Listing
February 2019
12 Reads

Risk perception before and after presymptomatic genetic testing for Huntington's disease: Not always what one might expect.

Mol Genet Genomic Med 2018 11 4;6(6):1140-1147. Epub 2018 Nov 4.

Berman Institute of Bioethics, Johns Hopkins University, Baltimore, Maryland.

Background: In 1983, Huntington's disease (HD) was the first genetic disease mapped using DNA polymorphisms. Shortly thereafter, presymptomatic genetic testing for HD began in the context of two research studies. One of these trials was at the Johns Hopkins University Huntington's Disease Center. Read More

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http://dx.doi.org/10.1002/mgg3.494DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305678PMC
November 2018
13 Reads

Variation in TMEM106B in chronic traumatic encephalopathy.

Acta Neuropathol Commun 2018 Nov 4;6(1):115. Epub 2018 Nov 4.

Boston University Alzheimer's Disease and CTE Center, Boston University School of Medicine, 72 E Concord Street, B7800, Boston, MA, 02118, USA.

The genetic basis of chronic traumatic encephalopathy (CTE) is poorly understood. Variation in transmembrane protein 106B (TMEM106B) has been associated with enhanced neuroinflammation during aging and with TDP-43-related neurodegenerative disease, and rs3173615, a missense coding SNP in TMEM106B, has been implicated as a functional variant in these processes. Neuroinflammation and TDP-43 pathology are prominent features in CTE. Read More

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https://actaneurocomms.biomedcentral.com/articles/10.1186/s4
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http://dx.doi.org/10.1186/s40478-018-0619-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6215686PMC
November 2018
31 Reads

Classification of Gait Patterns in Patients with Neurodegenerative Disease Using Adaptive Neuro-Fuzzy Inference System.

Comput Math Methods Med 2018 30;2018:9831252. Epub 2018 Sep 30.

Institute of Intelligent Machines, Chinese Academy of Sciences, Hefei 230031, China.

A common feature that is typical of the patients with neurodegenerative (ND) disease is the impairment of motor function, which can interrupt the pathway from cerebrum to the muscle and thus cause movement disorders. For patients with amyotrophic lateral sclerosis disease (ALS), the impairment is caused by the loss of motor neurons. While for patients with Parkinson's disease (PD) and Huntington's disease (HD), it is related to the basal ganglia dysfunction. Read More

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https://www.hindawi.com/journals/cmmm/2018/9831252/
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http://dx.doi.org/10.1155/2018/9831252DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186329PMC
February 2019
11 Reads

Origin of the Autophagosome Membrane in Mammals.

Biomed Res Int 2018 24;2018:1012789. Epub 2018 Sep 24.

Xiyuan Hospital of China Academy of Chinese Medical Sciences, Beijing 100091, China.

Autophagy begins with the nucleation of phagophores, which then expand to give rise to the double-membrane autophagosomes. Autophagosomes ultimately fuse with lysosomes, where the cytosolic cargoes are degraded. Accumulation of autophagosomes is a hallmark of autophagy and neurodegenerative disorders including Alzheimer's and Huntington's disease. Read More

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http://dx.doi.org/10.1155/2018/1012789DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174804PMC
January 2019
8 Reads

C9orf72 Repeat Expansion Frequency among Patients with Huntington Disease Genetic Testing.

Neurodegener Dis 2018 Oct 18;18(5-6):239-253. Epub 2018 Oct 18.

Division of Human Genetics and UC Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Background: European studies identified the C9orf72 repeat expansion as the most frequent genetic alteration in patients with Huntington disease (HD)-like phenotypes but negative HD genetic testing.

Objective: To investigate C9orf72 repeat expansion frequency in individuals tested for HD in a North American tertiary referral laboratory.

Methods: Three hundred and seventy-three cases (115 positive and 258 negative for HD) were evaluated by genotyping PCR, with follow-up Southern blot and 5' repeat methylation status assessment by combined repeat-primed and methylation-specific PCR in a subset. Read More

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https://www.karger.com/Article/FullText/492499
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http://dx.doi.org/10.1159/000492499DOI Listing
October 2018
10 Reads

Selection of Reference Regions to Model Neurodegeneration in Huntington Disease by 18F-FDG PET/CT Using Imaging and Clinical Parameters.

Clin Nucl Med 2019 01;44(1):e1-e5

Nuclear Medicine Department, Hospital de la Santa Creu i Sant Pau, Autonomous University of Barcelona.

Objective: Normalization to an appropriate reference region in F-FDG PET imaging may enhance diagnostic performance in Huntington disease (HD). We aimed to identify stable brain areas that could be used to model neurometabolic degeneration in HD correlating imaging (SUVrvalues at the basal ganglia [BBGG]) and clinical parameters (disease burden score [DBS]).

Materials And Methods: We performed brain F-FDG PET/CT in 38 manifest HD patients (meanage ± SD, 54 ± 14. Read More

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http://Insights.ovid.com/crossref?an=00003072-900000000-9741
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http://dx.doi.org/10.1097/RLU.0000000000002329DOI Listing
January 2019
11 Reads

Modulation of AMPA receptor surface diffusion restores hippocampal plasticity and memory in Huntington's disease models.

Nat Commun 2018 10 15;9(1):4272. Epub 2018 Oct 15.

Interdisciplinary Institute for Neuroscience, University of Bordeaux, Bordeaux, 33076, France.

Impaired hippocampal synaptic plasticity contributes to cognitive impairment in Huntington's disease (HD). However, the molecular basis of such synaptic plasticity defects is not fully understood. Combining live-cell nanoparticle tracking and super-resolution imaging, we show that AMPAR surface diffusion, a key player in synaptic plasticity, is disturbed in various rodent models of HD. Read More

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http://dx.doi.org/10.1038/s41467-018-06675-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6189172PMC
October 2018
12 Reads

Altered Levels of Long NcRNAs Meg3 and Neat1 in Cell And Animal Models Of Huntington's Disease.

RNA Biol 2018 26;15(10):1348-1363. Epub 2018 Oct 26.

b Crystallography and Molecular Biology Division , Saha Institute of Nuclear Physics, HBNI , Kolkata , India.

Altered expression levels of protein-coding genes and microRNAs have been implicated in the pathogenesis of Huntington's disease (HD). The involvement of other ncRNAs, especially long ncRNAs (lncRNA), is being realized recently and the related knowledge is still rudimentary. Using small RNA sequencing and PCR arrays we observed perturbations in the levels of 12 ncRNAs in HD mouse brain, eight of which had human homologs. Read More

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http://dx.doi.org/10.1080/15476286.2018.1534524DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6284602PMC
January 2019
5 Reads

Repeat length variations in ATXN1 and AR modify disease expression in Alzheimer's disease.

Neurobiol Aging 2019 Jan 15;73:230.e9-230.e17. Epub 2018 Sep 15.

Departments of Neurology, Leiden University Medical Centre, Leiden, the Netherlands; German Center for Neurodegenerative Diseases (DZNE), Bonn, Germany.

Genomewide association studies (GWASs) have contributed greatly to unraveling the genetic basis of Alzheimer's disease (AD). However, a large amount of "missing heritability" remains. In this exploratory study, we investigated the effect of cytosine-adenine-guanine (CAG) repeats in polyglutamine disease-associated genes (PDAGs) on the risk of AD and its expression. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2018.09.007DOI Listing
January 2019
3 Reads

Quinolinic Acid-Induced Huntington Disease-Like Symptoms Mitigated by Potent Free Radical Scavenger Edaravone-a Pilot Study on Neurobehavioral, Biochemical, and Histological Approach in Male Wistar Rats.

J Mol Neurosci 2018 Nov 3;66(3):322-341. Epub 2018 Oct 3.

Department of Medical Biochemistry, Dr. ALM Post Graduate Institute of Basic Medical Sciences, University of Madras, Taramani Campus, Chennai, Tamil Nadu, 600 113, India.

In this study, we demonstrated for the first time the neuroprotective role of edaravone (Eda) (5 and 10 mg/kg b.w.), a potent free radical scavenger against the unilateral stereotaxic induction of quinolinic acid (QA) (300 nm/4 μl saline)-induced Huntington disease (HD)-like symptoms in behavioral, biochemical, and histological features in male Wistar rats striatum. Read More

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http://dx.doi.org/10.1007/s12031-018-1168-1DOI Listing
November 2018
1 Read

Deubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's disease.

Nat Commun 2018 09 28;9(1):3191. Epub 2018 Sep 28.

Center for Systems and Therapeutics & Taube/Koret Center for Neurodegenerative Disease, Gladstone Institutes, 1650 Owens St., San Francisco, CA, 94158, USA.

Huntington's disease is a progressive neurodegenerative disorder caused by polyglutamine-expanded mutant huntingtin (mHTT). Here, we show that the deubiquitinase Usp12 rescues mHTT-mediated neurodegeneration in Huntington's disease rodent and patient-derived human neurons, and in Drosophila. The neuroprotective role of Usp12 may be specific amongst related deubiquitinases, as the closely related homolog Usp46 does not suppress mHTT-mediated toxicity. Read More

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http://dx.doi.org/10.1038/s41467-018-05653-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6162324PMC
September 2018
14 Reads
10.740 Impact Factor

Connectomic imaging reveals Huntington-related pathological and pharmaceutical effects in a mouse model.

NMR Biomed 2018 12 27;31(12):e4007. Epub 2018 Sep 27.

Singapore BioImaging Consortium, Agency for Science, Technology and Research, Singapore, Singapore.

Recent studies suggest that neurodegenerative diseases could affect brain structure and function in disease-specific network patterns; however, how spontaneous activity affects structural covariance network (SC) is not clear. We hypothesized that hyper-excitability in Huntington disease (HD) disrupts the coordinated structural and functional connectivity, and treatment with memantine helps to reduce excitotoxicity and normalize the connectivity. MRI was conducted to measure somatosensory activation, resting-state functional-connectivity (rsFC), SC, amplitude of low frequency fluctuation (ALFF) and ALFF covariance (ALFFC) in the YAC128 mouse model of HD. Read More

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http://dx.doi.org/10.1002/nbm.4007DOI Listing
December 2018
1 Read

Quantitative Measurements of Motor Function in Alzheimer's Disease, Frontotemporal Dementia, and Dementia with Lewy Bodies: A Proof-of-Concept Study.

Dement Geriatr Cogn Disord 2018 26;46(3-4):168-179. Epub 2018 Sep 26.

Danish Dementia Research Centre, Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen,

Background: This study examines the efficacy of using quantitative measurements of motor dysfunction, compared to clinical ratings, in Alzheimer's disease (AD), frontotemporal dementia (FTD), and dementia with Lewy bodies (DLB).

Methods: In this cross-sectional study, 49 patients with a diagnosis of AD (n = 17), FTD (n = 19), or DLB (n = 13) were included and underwent cognitive testing, clinical motor evaluation, and quantitative motor tests: pronation/supination hand tapping, grasping and lifting, and finger and foot tapping.

Results: Our results revealed significantly higher Q-Motor values in pronation/supination and in grip lift force assessment in AD, FTD, and DLB compared to healthy controls (HC). Read More

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http://dx.doi.org/10.1159/000492860DOI Listing
January 2019
3 Reads

Studying Huntington's Disease in Yeast: From Mechanisms to Pharmacological Approaches.

Front Mol Neurosci 2018 4;11:318. Epub 2018 Sep 4.

Institute of Molecular Biosciences, University of Graz, Graz, Austria.

Huntington's disease (HD) is a neurodegenerative disorder that leads to progressive neuronal loss, provoking impaired motor control, cognitive decline, and dementia. So far, HD remains incurable, and available drugs are effective only for symptomatic management. HD is caused by a mutant form of the huntingtin protein, which harbors an elongated polyglutamine domain and is highly prone to aggregation. Read More

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http://dx.doi.org/10.3389/fnmol.2018.00318DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131589PMC
September 2018
3 Reads

The role of microglia in processing and spreading of bioactive tau seeds in Alzheimer's disease.

J Neuroinflammation 2018 Sep 18;15(1):269. Epub 2018 Sep 18.

Alzheimer's Disease Research Laboratory, Department of Neurology, Mass General Institute for Neurodegenerative Disease, Massachusetts General Hospital and Harvard Medical School, 114 16th Street, Charlestown, MA, 02129, USA.

Background: Misfolding of microtubule-associated protein tau (MAPT) within neurons into neurofibrillary tangles is an important pathological feature of Alzheimer's disease (AD). Tau pathology correlates with cognitive decline in AD and follows a stereotypical anatomical course; several recent studies indicate that tau pathology spreads inter-neuronally via misfolded tau "seeds." Previous research has focused on neurons as the source of these tau seeds. Read More

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http://dx.doi.org/10.1186/s12974-018-1309-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6145371PMC
September 2018
18 Reads

Frequency and association of mitochondrial genetic variants with neurological disorders.

Mitochondrion 2018 Sep 13. Epub 2018 Sep 13.

Experimental Multiuser Laboratory (LEM), Graduate Program in Health Sciences (PPGCS), School of Medicine (PPGCS), Pontifícia Universidade Católica do Paraná (PUCPR), Curitiba, Paraná 80215-901, Brazil; Lico Kaesemodel Institute (ILK), Curitiba, Paraná 80240-000, Brazil. Electronic address:

Mitochondria are small cytosolic organelles and the main source of energy production for the cells, especially in the brain. This organelle has its own genome, the mitochondrial DNA (mtDNA), and genetic variants in this molecule can alter the normal energy metabolism in the brain, contributing to the development of a wide assortment of Neurological Disorders (ND), including neurodevelopmental syndromes, neurodegenerative diseases and neuropsychiatric disorders. These ND are comprised by a heterogeneous group of syndromes and diseases that encompass different cognitive phenotypes and behavioral disorders, such as autism, Asperger's syndrome, pervasive developmental disorder, attention deficit hyperactivity disorder, Huntington disease, Leigh Syndrome and bipolar disorder. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15677249183011
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http://dx.doi.org/10.1016/j.mito.2018.09.005DOI Listing
September 2018
10 Reads

Repeat-associated non-ATG (RAN) translation.

J Biol Chem 2018 Oct 13;293(42):16127-16141. Epub 2018 Sep 13.

From the Center for NeuroGenetics,

Microsatellite expansions cause more than 40 neurological disorders, including Huntington's disease, myotonic dystrophy, and C9ORF72 amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD). These repeat expansion mutations can produce epeat-ssociated on-ATG (RAN) proteins in all three reading frames, which accumulate in disease-relevant tissues. There has been considerable interest in RAN protein products and their downstream consequences, particularly for the dipeptide proteins found in ALS/FTD. Read More

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http://dx.doi.org/10.1074/jbc.R118.003237DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6200949PMC
October 2018
2 Reads

Executive impairment is associated with unawareness of neuropsychiatric symptoms in premanifest and early Huntington's disease.

Neuropsychology 2018 Nov 13;32(8):958-965. Epub 2018 Sep 13.

Monash Institute of Cognitive and Clinical Neurosciences, School of Psychological Sciences, Monash University.

Objective: Unawareness of neuropsychiatric symptoms appears to be common in Huntington's disease (HD), but the clinical correlates of unawareness are unclear. Identifying predictors of unawareness is important for improving diagnosis of neuropsychiatric symptoms, and cognitive impairment, specifically executive impairment, may be a potential important predictor of unawareness. The authors examined whether unawareness of neuropsychiatric symptoms is more common in early HD compared to premanifest HD, and whether executive task performance was associated with awareness, independent of demographic, motor or mood variables. Read More

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http://dx.doi.org/10.1037/neu0000479DOI Listing
November 2018
19 Reads

Effect of Regulatory Network of Exosomes and microRNAs on Neurodegenerative Diseases.

Chin Med J (Engl) 2018 Sep;131(18):2216-2225

Department of Environmental Engineering, College of Environmental Science and Engineering, China West Normal University, Nanchong, Sichuan 637009, China.

Objective: A comprehensive review of the network regulation of exosomes and microRNAs (miRNAs) in neurodegenerative diseases was done, centering on the mechanism of the formation of exosomes and miRNAs and the sorting mechanism of exosomal miRNAs, with the aim to provide a theoretical basis in the search of biomarkers and the treatment of neurodegenerative diseases.

Data Sources: The comprehensive search used online literature databases including NCBI PubMed, Web of Science, Google Scholar, and Baidu Scholar.

Study Selection: The study selection was based on the following keywords: exosomes, miRNAs, central nervous system (CNS), and neurodegenerative diseases. Read More

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http://www.cmj.org/text.asp?2018/131/18/2216/240817
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http://dx.doi.org/10.4103/0366-6999.240817DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6144844PMC
September 2018
10 Reads

Antidepressant Effects of Probucol on Early-Symptomatic YAC128 Transgenic Mice for Huntington's Disease.

Neural Plast 2018 14;2018:4056383. Epub 2018 Aug 14.

Department of Morphological Sciences, Center of Biological Sciences, Universidade Federal de Santa Catarina, 88040-900 Florianópolis, SC, Brazil.

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by a trinucleotide expansion in the gene, resulting in an extended polyglutamine tract in the protein huntingtin. HD is traditionally viewed as a movement disorder, but cognitive and neuropsychiatric symptoms also contribute to the clinical presentation. Depression is one of the most common psychiatric disturbances in HD, present even before manifestation of motor symptoms. Read More

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http://dx.doi.org/10.1155/2018/4056383DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6112232PMC
November 2018
2 Reads

Metformin reverses early cortical network dysfunction and behavior changes in Huntington's disease.

Elife 2018 09 4;7. Epub 2018 Sep 4.

Institute of Pathophysiology, Focus Program Translational Neurosciences, University Medical Center, Mainz, Germany.

Catching primal functional changes in early, 'very far from disease onset' (VFDO) stages of Huntington's disease is likely to be the key to a successful therapy. Focusing on VFDO stages, we assessed neuronal microcircuits in premanifest Hdh150 knock-in mice. Employing two-photon Ca imaging, we revealed an early pattern of circuit dysregulation in the visual cortex - one of the first regions affected in premanifest Huntington's disease - characterized by an increase in activity, an enhanced synchronicity and hyperactive neurons. Read More

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http://dx.doi.org/10.7554/eLife.38744DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6156080PMC
September 2018
3 Reads

Early postnatal behavioral, cellular, and molecular changes in models of Huntington disease are reversible by HDAC inhibition.

Proc Natl Acad Sci U S A 2018 09 27;115(37):E8765-E8774. Epub 2018 Aug 27.

Department of Experimental Therapy, Friedrich-Alexander-University, 91054 Erlangen, Germany;

Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by expanded CAG repeats in the gene (). Although mutant HTT is expressed during embryonic development and throughout life, clinical HD usually manifests later in adulthood. A number of studies document neurodevelopmental changes associated with mutant , but whether these are reversible under therapy remains unclear. Read More

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http://www.pnas.org/lookup/doi/10.1073/pnas.1807962115
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http://dx.doi.org/10.1073/pnas.1807962115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6140493PMC
September 2018
6 Reads
9.810 Impact Factor

Auditory time perception in Huntington's disease.

Neuropsychologia 2018 10 22;119:247-252. Epub 2018 Aug 22.

Swiss Huntington's Disease Centre, Neurozentrum Siloah, Gümligen, Switzerland; Department of Neurology, University of Bern, Switzerland. Electronic address:

Background: Huntington's disease (HD) is characterized by early involvement of the striatum. It affects the pace of repetitive motor activity, as motor timing depends on basal ganglia activity. However, data are lacking on the impact of this process on auditory time perception in motor non-affected gene carriers. Read More

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http://dx.doi.org/10.1016/j.neuropsychologia.2018.08.018DOI Listing
October 2018
1 Read

Daring to hope.

Authors:
Meredith Wadman

Science 2018 Aug 23;361(6404):742-746. Epub 2018 Aug 23.

Vancouver, Canada.

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http://www.sciencemag.org/lookup/doi/10.1126/science.361.640
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http://dx.doi.org/10.1126/science.361.6404.742DOI Listing
August 2018
3 Reads

Current understanding of Alzheimer's disease diagnosis and treatment.

F1000Res 2018 31;7. Epub 2018 Jul 31.

Department of Neurology, Boston VA Hospital, 150 South Huntington Street, Jamaica Plain, MA, 02130, USA.

Alzheimer's disease is the most common cause of dementia worldwide, with the prevalence continuing to grow in part because of the aging world population. This neurodegenerative disease process is characterized classically by two hallmark pathologies: β-amyloid plaque deposition and neurofibrillary tangles of hyperphosphorylated tau. Diagnosis is based upon clinical presentation fulfilling several criteria as well as fluid and imaging biomarkers. Read More

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http://dx.doi.org/10.12688/f1000research.14506.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6073093PMC
July 2018
3 Reads

Differential changes to D1 and D2 medium spiny neurons in the 12-month-old Q175+/- mouse model of Huntington's Disease.

PLoS One 2018 17;13(8):e0200626. Epub 2018 Aug 17.

Department of Anatomy & Neurobiology, Boston University School of Medicine, Boston, Massachusetts.

Huntington's Disease (HD) is an autosomal dominant, progressive neurodegenerative disorder caused by deleterious expansion of CAG repeats in the Huntingtin gene and production of neurotoxic mutant Huntingtin protein (mHTT). The key pathological feature of HD is a profound degeneration of the striatum and a loss of cortical volume. The initial loss of indirect pathway (D2) medium spiny neuron (MSN) projections in early stages of HD, followed by a loss of direct pathway (D1) projections in advanced stages has important implications for the trajectory of motor and cognitive dysfunction in HD, but is not yet understood. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0200626PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6097649PMC
January 2019
2 Reads

Guarana () Extract Protects Models for Alzheimer Disease and Huntington Disease through Activation of Antioxidant and Protein Degradation Pathways.

Oxid Med Cell Longev 2018 4;2018:9241308. Epub 2018 Jul 4.

Núcleo de Pesquisa em Ciências Biológicas, Universidade Federal de Ouro Preto, Ouro Preto, MG, Brazil.

Guarana () is largely consumed in Brazil in high energy drinks and dietary supplements because of its stimulant activity on the central nervous system. Although previous studies have indicated that guarana has some protective effects in Parkinson's (PD), Alzheimer's (AD), and Huntington's (HD) disease models, the underlying mechanisms are unknown. Here, we investigated the protective effects of guarana hydroalcoholic extract (GHE) in models of HD and AD. Read More

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https://www.hindawi.com/journals/omcl/2018/9241308/
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http://dx.doi.org/10.1155/2018/9241308DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6079341PMC
December 2018
15 Reads

Promoting the clearance of neurotoxic proteins in neurodegenerative disorders of ageing.

Nat Rev Drug Discov 2018 Sep 17;17(9):660-688. Epub 2018 Aug 17.

Centre for Therapeutic Innovation in Neuropsychiatry, IDR Servier, 78290 Croissy sur Seine, France.

Neurodegenerative disorders of ageing (NDAs) such as Alzheimer disease, Parkinson disease, frontotemporal dementia, Huntington disease and amyotrophic lateral sclerosis represent a major socio-economic challenge in view of their high prevalence yet poor treatment. They are often called 'proteinopathies' owing to the presence of misfolded and aggregated proteins that lose their physiological roles and acquire neurotoxic properties. One reason underlying the accumulation and spread of oligomeric forms of neurotoxic proteins is insufficient clearance by the autophagic-lysosomal network. Read More

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http://www.nature.com/doifinder/10.1038/nrd.2018.109
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http://dx.doi.org/10.1038/nrd.2018.109DOI Listing
September 2018
38 Reads

New Yes/No Recognition Memory Analysis on the California Verbal Learning Test-3: Clinical Utility in Alzheimer's and Huntington's Disease.

J Int Neuropsychol Soc 2018 Sep 16;24(8):833-841. Epub 2018 Aug 16.

1San Diego State University/University of California San Diego Joint Doctoral Program in Clinical Psychology,San Diego/La Jolla,California.

Objectives: The third edition of the California Verbal Learning Test (CVLT-3) includes a new index termed List A versus Novel/Unrelated recognition discriminability (RD) on the Yes/No Recognition trial. Whereas the Total RD index incorporates false positive (FP) errors associated with all distractors (including List B and semantically related items), the new List A versus Novel/Unrelated RD index incorporates only FP errors associated with novel, semantically unrelated distractors. Thus, in minimizing levels of source and semantic interference, the List A versus Novel/Unrelated RD index may yield purer assessments of yes/no recognition memory independent of vulnerability to source memory difficulties or semantic confusion, both of which are often seen in individuals with primarily frontal-system dysfunction (e. Read More

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http://dx.doi.org/10.1017/S1355617718000474DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6170690PMC
September 2018

The Tiny for the Biggest Answers in Huntington's Disease.

Int J Mol Sci 2018 Aug 14;19(8). Epub 2018 Aug 14.

Instituto de Bioquímica y Microbiología, Facultad de Ciencias, Universidad Austral de Chile, Valdivia 5090000, Chile.

The average life expectancy for humans has increased over the last years. However, the quality of the later stages of life is low and is considered a public health issue of global importance. Late adulthood and the transition into the later stage of life occasionally leads to neurodegenerative diseases that selectively affect different types of neurons and brain regions, producing motor dysfunctions, cognitive impairment, and psychiatric disorders that are progressive, irreversible, without remission periods, and incurable. Read More

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http://dx.doi.org/10.3390/ijms19082398DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6121572PMC
August 2018
2 Reads

CYP46A1 protects against NMDA-mediated excitotoxicity in Huntington's disease: Analysis of lipid raft content.

Biochimie 2018 Oct 11;153:70-79. Epub 2018 Aug 11.

Institut du Cerveau et de la Moelle épinière (ICM), UMR-S975, Université Pierre et Marie Curie-Paris 6, Hôpital de la Salpêtrière, Paris, France. Electronic address:

Huntington's Disease (HD) is an autosomal dominant neurodegenerative disease caused by abnormal polyglutamine expansion in huntingtin (mHtt) protein leading to degeneration of striatal neurons. Excitotoxicity, consecutive to overstimulation of N-methyl d-aspartate receptors (NMDARs) has a pivotal role in many neurological disorders including HD. Mutant Htt causes enhanced NMDA sensitivity, alteration of NMDAR expression and localization in neurons. Read More

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http://dx.doi.org/10.1016/j.biochi.2018.07.019DOI Listing
October 2018
21 Reads

Complex spatial and temporally defined myelin and axonal degeneration in Huntington disease.

Neuroimage Clin 2018 19;20:236-242. Epub 2018 Feb 19.

Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

Although much prior work has focused on the basal ganglia and cortical pathology that defines Huntington's disease (HD), recent studies have also begun to characterize cerebral white matter damage (Rosas et al., 2006; Dumas et al., 2012; Poudel et al. Read More

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http://dx.doi.org/10.1016/j.nicl.2018.01.029DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6078048PMC
January 2019
19 Reads

Effects of multidisciplinary therapy on physical function in Huntington's disease.

Acta Neurol Scand 2018 Dec 30;138(6):500-507. Epub 2018 Jul 30.

School of Medical and Health Sciences, Edith Cowan University, Perth, Western Australia, Australia.

Objective: The primary objective of this trial was to evaluate the effects of outpatient multidisciplinary therapy, compared to usual care, on measures of physical function and muscle strength in patients with manifest Huntington's disease (HD).

Methods: Twenty-two patients with clinically verified HD were randomized to receive 36 weeks of outpatient multidisciplinary therapy or usual care. Outpatient multidisciplinary therapy comprised 9 months of supervised exercise, cognitive therapy and self-directed home-based exercise. Read More

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http://dx.doi.org/10.1111/ane.13002DOI Listing
December 2018
6 Reads

Brain-Derived Neurotrophic Factor (BDNF) and Serotonin Transporter (SERT) in Platelets of Patients with Mild Huntington's Disease: Relationships with Social Cognition Symptoms.

Arch Ital Biol 2018 07;156(1-2):27-39

Department of Clinical and Experimental Medicine, University of Pisa, 56126 Pisa, Italy - Email:

Deficits in social-cognition processing have been identified during early stages of Huntington Disease (HD), attracting interest on their relevance as possible predictors of  neurodegenerative progression. Since the neurotrophin Brain-Derived Neurotrophic Factor (BDNF) and the serotonin (5-HT) transporter (SERT) are known to modulate human adaptive behavior, we appraised these two proteins in mild-HD using blood platelets, with the aim at finding relationships with cognitive/psychosocial skills. Thirteen gene positive and symptomatic patients (9M/4W, HD-stage II, age> 40y) together 11 gender/age matched controls without a concurrent diagnosis of psychiatric disorders, underwent a blood test to determine BDNF storage and membrane-bound SERT in platelets by an ELISA immune-enzyme dosage and [3H]-paroxetine ([3H]-PAR) binding, respectively. Read More

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http://dx.doi.org/10.12871/00039829201813DOI Listing
July 2018
9 Reads

The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington's disease patients.

Nat Commun 2018 07 23;9(1):2886. Epub 2018 Jul 23.

Institute for Genetics and Cologne Excellence Cluster for Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne, Joseph Stelzmann Strasse 26, 50931, Cologne, Germany.

Induced pluripotent stem cells (iPSCs) undergo unlimited self-renewal while maintaining their potential to differentiate into post-mitotic cells with an intact proteome. As such, iPSCs suppress the aggregation of polyQ-expanded huntingtin (HTT), the mutant protein underlying Huntington's disease (HD). Here we show that proteasome activity determines HTT levels, preventing polyQ-expanded aggregation in iPSCs from HD patients (HD-iPSCs). Read More

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http://dx.doi.org/10.1038/s41467-018-05320-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6056416PMC
July 2018
3 Reads

Apathy and atrophy of subcortical brain structures in Huntington's disease: A two-year follow-up study.

Neuroimage Clin 2018 27;19:66-70. Epub 2018 Mar 27.

Leiden University Medical Center, Department of Neurology, Leiden, The Netherlands.

Background: Huntington's disease (HD) is characterized by motor and behavioral symptoms, and cognitive decline. HD gene carriers and their caregivers report the behavioral and cognitive symptoms as the most burdensome. Apathy is the most common behavioral symptom of HD and is related to clinical measures of disease progression, like functional capacity. Read More

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http://dx.doi.org/10.1016/j.nicl.2018.03.033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6051315PMC
January 2019
8 Reads

Prevalence of Aging, Dementia, and Multimorbidity in Older Adults With Down Syndrome.

JAMA Neurol 2018 Nov;75(11):1399-1406

Global Brain Health Institute, University of California, San Francisco.

Importance: As the life expectancy of people with Down syndrome (DS) has markedly increased over the past decades, older adults with DS may be experiencing a higher incidence of aging conditions. In addition to longevity, the amyloid precursor protein gene located on chromosome 21 places individuals with DS at a high risk for developing Alzheimer disease. Yet, few studies have determined prevalence of dementia and comorbidities among older people with DS. Read More

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http://archneur.jamanetwork.com/article.aspx?doi=10.1001/jam
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http://dx.doi.org/10.1001/jamaneurol.2018.2210DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6248113PMC
November 2018
18 Reads

Human cellular models of medium spiny neuron development and Huntington disease.

Authors:
Monika M Golas

Life Sci 2018 Sep 18;209:179-196. Epub 2018 Jul 18.

Department of Biomedicine, Aarhus University, Wilhelm Meyers Alle 3, Building 1233, DK-8000 Aarhus C, Denmark; Department of Human Genetics, Hannover Medical School, Carl-Neuberg-Str. 1, 30625 Hannover, Germany. Electronic address:

The loss of gamma-aminobutyric acid (GABA)-ergic medium spiny neurons (MSNs) in the striatum is the hallmark of Huntington disease (HD), an incurable neurodegenerative disorder characterized by progressive motor, psychiatric, and cognitive symptoms. Transplantation of MSNs or their precursors represents a promising treatment strategy for HD. In initial clinical trials in which HD patients received fetal neurografts directly into the striatum without a pretransplant cell-differentiation step, some patients exhibited temporary benefits. Read More

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http://dx.doi.org/10.1016/j.lfs.2018.07.030DOI Listing
September 2018
3 Reads

Huntington's disease: the coming of age.

J Genet 2018 Jul;97(3):649-664

Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA.

Huntington's disease (HD) is caused due to an abnormal expansion of polyglutamine repeats in the first exon of huntingtin gene. The mutation in huntingtin causes abnormalities in the functioning of protein, leading to deleterious effects ultimately to the demise of specific neuronal cells.The disease is inherited in an autosomal dominant manner and leads to a plethora of neuropsychiatric behaviour and neuronal cell death mainly in striatal and cortical regions of the brain, eventually leading to death of the individual. Read More

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July 2018
23 Reads

Neuroprotective activity of tetramethylpyrazine against 3-nitropropionic acid induced Huntington's disease-like symptoms in rats.

Biomed Pharmacother 2018 Sep 22;105:1254-1268. Epub 2018 Jun 22.

Department of Pharmaceutical Chemistry, University College of Pharmaceutical Sciences, Acharya Nagarjuna University, India.

Huntington's disease (HD) is an autosomal neurodegenerative disease characterized by chorea, dystonia, motor ataxia, cognitive decline and psychiatric disorders with gradual loss of nerve cells and has no existing cure for the disease. In the present study, a mitochondrial toxin, 3-nitropropionic acid (3-NP) is used to induce HD like symptoms in rats. Tetramethylpyrazine is one of the active ingredients of Chuan Xiong which was reported to have neurotrophic and neuroprotective activities. Read More

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http://dx.doi.org/10.1016/j.biopha.2018.06.079DOI Listing
September 2018
6 Reads

Fibroblast Growth Factor 9 Suppresses Striatal Cell Death Dominantly Through ERK Signaling in Huntington's Disease.

Cell Physiol Biochem 2018 18;48(2):605-617. Epub 2018 Jul 18.

Taiwan International Graduate Program in Interdisciplinary Neuroscience, National Cheng Kung University and Academia Sinica, Taipei, Taiwan.

Background/aims: Huntington's disease (HD) is a heritable neurodegenerative disorder, and there is no cure for HD to date. A type of fibroblast growth factor (FGF), FGF9, has been reported to play prosurvival roles in other neurodegenerative diseases, such as Parkinson's disease and Alzheimer's disease. However, the effects of FGF9 on HD is still unknown. Read More

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http://dx.doi.org/10.1159/000491889DOI Listing
September 2018
13 Reads

Bioinformatic analysis of microRNA expression in Huntington's disease.

Mol Med Rep 2018 Sep 29;18(3):2857-2865. Epub 2018 Jun 29.

Department of Neurology, Shengjing Hospital of China Medical University, Shenyang, Liaoning 110004, P.R. China.

Huntington's disease (HD) is an inherited, progressive neurodegenerative disease caused by a CAG expansion in the Huntingtin (HTT) gene and various dysfunctions of biological processes in HD have been proposed. Although monogenic, the exact pathogenesis of HD currently remains unclear. To identify the synergistic microRNA (miRNA) pattern in HD, the miRNA expression profile dataset GSE64977 and the gene expression profile dataset GSE64810 were downloaded. Read More

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http://dx.doi.org/10.3892/mmr.2018.9238DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6102687PMC
September 2018
8 Reads