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Huntington Disease

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NK cell-derived GM-CSF potentiates inflammatory arthritis and is negatively regulated by CIS.

Cynthia Louis, Fernando Souza-Fonseca-Guimaraes, Yuyan Yang, Damian D'Silva, Tobias Kratina, Laura Dagley, Soroor Hediyeh-Zadeh, Jai Rautela, Seth Lucian Masters, Melissa J Davis, Jeffrey J Babon, Bogoljub Ciric, Eric Vivier, Warren S Alexander, Nicholas D Huntington, Ian P Wicks,

Despite increasing recognition of the importance of GM-CSF in autoimmune disease, it remains unclear how GM-CSF is regulated at sites of tissue inflammation. Using GM-CSF fate reporter mice, we show that synovial NK cells produce GM-CSF in autoantibody-mediated inflammatory arthritis. Synovial NK cells promote a neutrophilic inflammatory cell infiltrate, and ... Read more >>

J. Exp. Med. (The Journal of experimental medicine)
[2020, 217(5):]

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The Phasor FLIM Method Reveals a Link Between a Change in Energy Metabolism and mHtt protein spread in Healthy Mammalian Cells when Co-cultured with Huntington Diseased Cells.

Sara Sameni, Run Zhang, Michelle A Digman,

Huntington Disease (HD) is a late-onset autosomal neurodegenerative disease characterized by the aggregations of mutant Huntingtin proteins (mHTT). A glutamine stretch (PolyQ) at the N-terminal of the Huntingtin protein is generated by the abnormal expansion of CAG trinucleotide repeats in exon 1 of the HTT gene. While the resulting polyQ ... Read more >>

Methods Appl Fluoresc (Methods and applications in fluorescence)
[2020, :]

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Huntingtin-Lowering Therapies for Huntington Disease: A Review of the Evidence of Potential Benefits and Risks.

Blair R Leavitt, Holly B Kordasiewicz, Scott A Schobel,

Huntington disease (HD) is caused by a cytosine-adenine-guanine trinucleotide repeat expansion in the huntingtin gene, HTT, that results in expression of variant (mutant) huntingtin protein (HTT). Therapeutic strategies that reduce HTT levels are currently being pursued to slow or stop disease progression in people with HD. These approaches are supported ... Read more >>

JAMA Neurol (JAMA neurology)
[2020, :]

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Pearl powder reduces sleep disturbance stress response through regulating proteomics in a rat model of sleep deprivation.

Meng Xia, Delun Huang, Yuangming Tong, Jiang Lin,

AIMS:This study aimed to explore whether pearl could help prevent cognitional morbidity and improve the metabolic processes of hippocampus. METHODS:Rats were divided into group of control (CTL), sleep deprivation (SD) and pearl powder (PP). The sleeplessness was introduced to all rats except control. Before and after administration with vehicle or ... Read more >>

J. Cell. Mol. Med. (Journal of cellular and molecular medicine)
[2020, :]

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Huntington's Disease Clinical Trials Corner: March 2020.

Filipe B Rodrigues, Edward J Wild,

In this edition of the Huntington's Disease Clinical Trials Corner we expand on the UniQure AMT-130 and on the Neurocrine Biosciences KINECT-HD trials, and list all currently registered and ongoing clinical trials in Huntington's disease. ... Read more >>

J Huntingtons Dis (Journal of Huntington's disease)
[2020, :]

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Patient-reported impact of symptoms in Huntington disease: PRISM-HD.

Alistair M Glidden, Elizabeth A Luebbe, Molly J Elson, Steven B Goldenthal, Christopher W Snyder, Christine E Zizzi, E Ray Dorsey, Chad R Heatwole,

OBJECTIVE:To determine the frequency and relative importance of symptoms experienced by adults with Huntington disease (HD) and to identify factors associated with a higher disease burden. METHODS:We performed 40 qualitative interviews (n = 20 with HD, n = 20 caregivers) and analyzed 2,082 quotes regarding the symptomatic burden of HD. ... Read more >>

Neurology (Neurology)
[2020, :]

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A comparison between the neurocognitive profile of Huntington Disease-Like 2 and Huntington Disease: Exploring the presence of double dissociations.

Aline Ferreira-Correia, David G Anderson, Kate Cockcroft, Amanda Krause,

Huntington Disease Like-2 (HDL2) is a rare autosomal dominant genetic disease caused by a mutation in the JPH3 gene. HDL2 is the Huntington Disease (HD) phenocopy that has the greatest clinical resemblance to HD. Both are characterized by movement, psychiatric and cognitive dysfunction, which progress to dementia. The present study ... Read more >>

Appl Neuropsychol Adult (Applied neuropsychology. Adult)
[2020, :1-11]

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A role for PGC-1α in transcription and excitability of neocortical and hippocampal excitatory neurons.

L J McMeekin, A F Bartley, A S Bohannon, E W Adlaf, T van Groen, S M Boas, S N Fox, P J Detloff, D K Crossman, L S Overstreet-Wadiche, J J Hablitz, L E Dobrunz, R M Cowell,

The transcriptional coactivator peroxisome proliferator-activated receptor γ coactivator-1α (PGC-1α) is a critical regulator of genes involved in neuronal metabolism, neurotransmission, and morphology. Reduced PGC-1α expression has been implicated in several neurological and psychiatric disorders. An understanding of PGC-1α's roles in different cell types will help determine the functional consequences of ... Read more >>

Neuroscience (Neuroscience)
[2020, :]

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Examining aggregates through the eyes of WDFY3/Alfy.

Leora Fox, Ai Yamamoto,

The role protein aggregates play in the pathogenesis of neurodegenerative diseases has been a question since their initial observation. In this autophagic punctum, we discuss our recent findings of how the selectivity scaffold/adaptor WDFY3/Alfy is required for the turnover of aggregated mutant HTT (huntingtin; mHTT) in the adult brain, and ... Read more >>

Autophagy (Autophagy)
[2020, 16(5):967-968]

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Stem Cells: A Review Encompassing the Literature with a Special Focus on the Side-Lined Miraculous Panacea; Pre-Morula Stem Cells.

Aryendu Saini, Rakesh Saini, Himanshu Bansode, Anurag Singh, Lalita Singh,

Stem cells are the undifferentiated cells in the body that possess the ability to differentiate and give rise to any type of cell in the body. In recent years, there has been a growing interest in therapy involving the stem cells as different treatment methods got developed. Depending on the ... Read more >>

Curr Stem Cell Res Ther (Current stem cell research & therapy)
[2020, :]

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Identification of Subtle Verbal Memory Deficits in Premanifest Huntington Disease Using the California Verbal Learning Test.

Heather M Holden, Savanna M Tierney, Lisa V Graves, Ilex Beltran-Najera, Steven Paul Woods, Chase M Snell, Dean C Delis, Jody Corey-Bloom, Paul E Gilbert,

BACKGROUND:Verbal memory impairment in individuals with Huntington disease (HD) is well-documented; however, the nature and extent of verbal memory impairment in individuals with premanifest HD (pre-HD) are less understood. OBJECTIVE:To evaluate verbal memory function in individuals with pre-HD by comparing their performance on the California Verbal Learning Test to that ... Read more >>

Cogn Behav Neurol (Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology)
[2020, 33(1):16-22]

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Piperine mitigates behavioral impairments and provides neuroprotection against 3-nitropropinoic acid-induced Huntington disease-like symptoms.

Mohd Salman, Heena Tabassum, Suhel Parvez,

Background: Piperine (PIP) is a powerful anti-oxidant and anti-inflammatory alkaloid which has been widely used in the treatment of various pathological conditions. However, few studies have clearly discussed the protective effects and potential mechanism of PIP in different neurological diseases. The aim of this study was to investigate the neuroprotective ... Read more >>

Nutr Neurosci (Nutritional neuroscience)
[2020, :1-10]

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Mutant HTT (huntingtin) impairs mitophagy in a cellular model of Huntington disease.

Sandra Franco-Iborra, Ainhoa Plaza-Zabala, Marta Montpeyo, David Sebastian, Miquel Vila, Marta Martinez-Vicente,

The precise degradation of dysfunctional mitochondria by mitophagy is essential for maintaining neuronal homeostasis. HTT (huntingtin) can interact with numerous other proteins and thereby perform multiple biological functions within the cell. In this study, we investigated the role of HTT during mitophagy and analyzed the impact of the expansion of ... Read more >>

Autophagy (Autophagy)
[2020, :1-18]

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Robust Markers and Sample Sizes for Multicenter Trials of Huntington Disease.

Peter A Wijeratne, Eileanoir B Johnson, Arman Eshaghi, Leon Aksman, Sarah Gregory, Hans J Johnson, Govinda R Poudel, Amrita Mohan, Cristina Sampaio, Nellie Georgiou-Karistianis, Jane S Paulsen, Sarah J Tabrizi, Rachael I Scahill, , Daniel C Alexander,

OBJECTIVE:The identification of sensitive biomarkers is essential to validate therapeutics for Huntington disease (HD). We directly compare structural imaging markers across the largest collective imaging HD dataset to identify a set of imaging markers robust to multicenter variation and to derive upper estimates on sample sizes for clinical trials in ... Read more >>

Ann. Neurol. (Annals of neurology)
[2020, :]

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An Optimization of AAV-82Q-Delivered Rat Model of Huntington's Disease.

Kyoung-Ha So, Jai Ho Choi, Jaisan Islam, Elina Kc, Hyeong Cheol Moon, So Yoon Won, Hyong Kyu Kim, Soochong Kim, Sang-Hwan Hyun, Young Seok Park,

Objective:No optimum genetic rat Huntington model both neuropathological using an adeno-associated virus (AAV-2) vector vector has been reported to date. We investigated whether direct infection of an AAV2 encoding a fragment of mutant huntingtin (AV2-82Q) into the rat striatum was useful for optimizing the Huntington rat model. Methods:We prepared ten ... Read more >>

J Korean Neurosurg Soc (Journal of Korean Neurosurgical Society)
[2020, :]

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Investigation of intermediate CAG alleles of the HTT in the general population of Rio de Janeiro, Brazil, in comparison with a sample of Huntington disease-affected families.

Thays A Apolinário, Iane Dos Santos da Silva, Luciana de Andrade Agostinho, Carmen L A Paiva,

BACKGROUND:Huntington disease (HD) (MIM: 143100) is a severe autosomal dominant neurodegenerative disease caused by the expansion of CAG trinucleotides (>35) in the HTT. OBJECTIVE:To investigate the frequency of intermediate CAG alleles (IAs) in individuals residing in Rio de Janeiro city with no familial history of HD (general population, GP) in ... Read more >>

Mol Genet Genomic Med (Molecular genetics & genomic medicine)
[2020, :e1181]

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Antisense oligonucleotides for neurodegeneration.

Blair R Leavitt, Sarah J Tabrizi,

Science (Science (New York, N.Y.))
[2020, 367(6485):1428-1429]

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Systematic review protocol: Quantitative susceptibility mapping of brain iron accumulation in neurodegenerative diseases.

Parsa Ravanfar, Samantha Loi, Tamsyn Van Rheenen, Ashley Bush, Patricia Desmond, Vanessa Cropley, Bradford Moffat, Dennis Velakoulis, Christos Pantelis,

Iron has been found to play an important role in neurodegeneration. Quantitative susceptibility mapping (QSM) is a relatively new, and the most accurate, MRI technique available for assessment of iron deposition in the brain. There is a rapidly growing number of studies using QSM to investigate brain iron distribution in ... Read more >>

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Dissection of prostate tumour, stroma and immune transcription reveals a key contribution by the microenvironment to disease progression

Stefano Mangiola, Patrick McCoy, Martin Modrak, Fernando Souza-Fonseca-Guimaraes, Daniel Blashki, Ryan Stuchbery, Michael Kerger, Ken Chow, Chayanica Nasa, Melanie Le Page, Natalie Lister, Simon Monard, Justin Peters, Phil Dundee, Anthony Costello, Bhupinder Pal, Nicholas Huntington, Niall Corcoran, Anthony Papenfuss, Christopher Hovens,

Abstract Background Prostate cancer is caused by genomic aberrations in normal epithelial cells, however clinical translation of findings from analyses of cancer cells alone has been very limited. A deeper understanding of the tumour microenvironment is needed to identify the key drivers of disease progression and reveal novel therapeutic opportunities. ... Read more >>

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Contractile Properties of Vaginal Tissue.

Alyssa Huntington, Kandace Donaldson, Raffaella De Vita,

The vagina is an important organ of the female reproductive system that has been largely under- studied in the field of biomechanics. In recent years, some research has been conducted to evaluate the mechanical properties of the vagina, but much has focused on characterizing the passive mechanical prop- erties. Because ... Read more >>

J Biomech Eng (Journal of biomechanical engineering)
[2020, :]

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Judgment in ABC case rules on confidentiality.

Jacqui Thornton,

Lancet (Lancet (London, England))
[2020, 395(10226):771-772]

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Deficit in motor skill consolidation-dependent synaptic plasticity at motor cortex to Dorso Lateral Striatum synapses in a mouse model of Huntington's disease.

Christelle Glangetas, Pedro Espinosa, Camilla Bellone,

Huntington's disease (HD) is a neurodegenerative disease notably characterized by progressive motor symptoms. Although the loss of Medium Spiny Neurons (MSNs) in the striatum has been associated with motor deficits, premanifest patients already present cognitive deficiencies and show early signs of motor disabilities. Here in a YAC128 HD mouse model, ... Read more >>

eNeuro (eNeuro)
[2020, :]

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Estimating disease onset from change points of markers measured with error.

Unkyung Lee, Raymond J Carroll, Karen Marder, Yuanjia Wang, Tanya P Garcia,

Huntington disease is an autosomal dominant, neurodegenerative disease without clearly identified biomarkers for when motor-onset occurs. Current standards to determine motor-onset rely on a clinician's subjective judgment that a patient's extrapyramidal signs are unequivocally associated with Huntington disease. This subjectivity can lead to error which could be overcome using an ... Read more >>

Biostatistics (Biostatistics (Oxford, England))
[2020, :]

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The dynamic mutation investigation and whole exome sequencing in a cohort of Chinese autosomal dominant cerebellar ataxia patients

Jianjun Wu, Fang Peng, Yue Zhang, Xin-Yue Zhou, Shuai-Qi Huang, Chen Chen, Zheng-Tong Ding, Jian Wang, Yi-Min Sun,

<title>Abstract</title> <p> Background Spinocerebellar ataxias (SCAs) are the autosomal dominant cerebellar ataxia (ADCA) with great clinical and genetic heterogeneity. Genetic testing will contribute to the final diagnosis. Methods A total of 204 Chinese ADCA patients were recruited and 190 had genetic testing. Dynamic mutations of SCA1, 2, 3, ... Read more >>

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Association Between Neurological Disorders and Death by Suicide in Denmark.

Annette Erlangsen, Egon Stenager, Yeates Conwell, Per Kragh Andersen, Keith Hawton, Michael Eriksen Benros, Merete Nordentoft, Elsebeth Stenager,

Importance:Neurological disorders have been linked to suicide, but the risk across a broad spectrum of neurological disorders remains to be assessed. Objectives:To examine whether people with neurological disorders die by suicide more often than other people and to assess for temporal associations. Design, Setting, and Participants:Nationwide, retrospective cohort study on ... Read more >>

JAMA (JAMA)
[2020, 323(5):444-454]

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