4.5 Review

Therapeutic Advances for Huntington's Disease

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Brain structure in juvenile-onset Huntington disease

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Targeting Huntingtin Expression in Patients with Huntington's Disease

Sarah J. Tabrizi et al.

NEW ENGLAND JOURNAL OF MEDICINE (2019)

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CRISPR-Cas9-Mediated Genome Editing Increases Lifespan and Improves Motor Deficits in a Huntington's Disease Mouse Model

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MOLECULAR THERAPY-NUCLEIC ACIDS (2019)

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Combination of stem cell and gene therapy ameliorates symptoms in Huntington's disease mice

In Ki Cho et al.

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Huntington's disease: a clinical review

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EUROPEAN JOURNAL OF NEUROLOGY (2018)

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Translation of MicroRNA-Based Huntingtin-Lowering Therapies from Preclinical Studies to the Clinic

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Effect of Trinucleotide Repeats in the Huntington's Gene on Intelligence

Jessica K. Lee et al.

EBIOMEDICINE (2018)

Article Neurosciences

Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease

Dagmar E. Ehrnhoefer et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2018)

Review Biotechnology & Applied Microbiology

Therapeutic approaches to Huntington disease: from the bench to the clinic

Nicholas S. Caron et al.

NATURE REVIEWS DRUG DISCOVERY (2018)

Review Neurosciences

Pridopidine: Overview of Pharmacology and Rationale for its Use in Huntington's Disease

Susanna Waters et al.

JOURNAL OF HUNTINGTONS DISEASE (2018)

Review Pharmacology & Pharmacy

Current Pharmacological Approaches to Reduce Chorea in Huntington's Disease

Emma M. Coppen et al.

Review Neurosciences

Huntington's Disease: Relationship Between Phenotype and Genotype

Yi-Min Sun et al.

MOLECULAR NEUROBIOLOGY (2017)

Article Biochemistry & Molecular Biology

MR-guided parenchymal delivery of adeno-associated viral vector serotype 5 in non-human primate brain

L. Samaranch et al.

GENE THERAPY (2017)

Article Medicine, Research & Experimental

CRISPR/Cas9-mediated gene editing ameliorates neurotoxicity in mouse model of Huntington's disease

Su Yang et al.

JOURNAL OF CLINICAL INVESTIGATION (2017)

Correction Clinical Neurology

Therapies targeting DNA and RNA in Huntington's disease (vol 16, pg 837, 2017)

E. J. Wild et al.

LANCET NEUROLOGY (2017)

Article Biotechnology & Applied Microbiology

CRISPR/Cas9 Editing of the Mutant Huntingtin Allele In Vitro and In Vivo

Alex Mas Monteys et al.

MOLECULAR THERAPY (2017)

Article Clinical Neurology

The CREST-E study of creatine for Huntington disease A randomized controlled trial

Steven M. Hersch et al.

NEUROLOGY (2017)

Article Neurosciences

Risk factors for the onset and progression of Huntington disease

Ting-Kuang Chao et al.

NEUROTOXICOLOGY (2017)

Review Neurosciences

RNAi mechanisms in Huntington's disease therapy: siRNA versus shRNA

Sebastian Aguiar et al.

TRANSLATIONAL NEURODEGENERATION (2017)

Article Clinical Neurology

A survey-based study identifies common but unrecognized symptoms in a large series of juvenile Huntington's disease

Amelia D. Moser et al.

NEURODEGENERATIVE DISEASE MANAGEMENT (2017)

Review Clinical Neurology

Therapies targeting DNA and RNA in Huntington's disease

Edward J. Wild et al.

LANCET NEUROLOGY (2017)

Review Neurosciences

Modern Genome Editing Technologies in Huntington's Disease Research

Tuyana B. Malankhanova et al.

JOURNAL OF HUNTINGTONS DISEASE (2017)

Review Biochemistry & Molecular Biology

mTOR Signaling in Growth, Metabolism, and Disease

Robert A. Saxton et al.

Article Neurosciences

Laquinimod decreases Bax expression and reduces caspase-6 activation in neurons

Dagmar E. Ehrnhoefer et al.

EXPERIMENTAL NEUROLOGY (2016)

Article Biochemistry & Molecular Biology

Pridopidine activates neuroprotective pathways impaired in Huntington Disease

Michal Geva et al.

HUMAN MOLECULAR GENETICS (2016)

Review Genetics & Heredity

The P42 peptide and Peptide-based therapies for Huntington's disease

Cecilia Marelli et al.

ORPHANET JOURNAL OF RARE DISEASES (2016)

Article Multidisciplinary Sciences

Systemic peptide-mediated oligonucleotide therapy improves long-term survival in spinal muscular atrophy

Suzan M. Hammond et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2016)

Article Biochemistry & Molecular Biology

Revisiting the Lamotrigine-Mediated Effect on Hippocampal GABAergic Transmission

Yu-Yin Huang et al.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2016)

Article Clinical Neurology

MIND diet associated with reduced incidence of Alzheimer's disease

Martha Clare Morris et al.

ALZHEIMERS & DEMENTIA (2015)

Article Pharmacology & Pharmacy

An exploratory double-blind, randomized clinical trial with selisistat, a SirT1 inhibitor, in patients with Huntington's disease

Sigurd D. Suessmuth et al.

BRITISH JOURNAL OF CLINICAL PHARMACOLOGY (2015)

Review Genetics & Heredity

Huntington's disease: An update of therapeutic strategies

Ashok Kumar et al.

Article Clinical Neurology

Ethyl-Eicosapentaenoic Acid Treatment in Huntington's Disease: A Placebo-Controlled Clinical Trial

Joaquim J. Ferreira et al.

MOVEMENT DISORDERS (2015)

Review Biochemistry & Molecular Biology

Therapeutic genome editing: prospects and challenges

David Benjamin Turitz Cox et al.

NATURE MEDICINE (2015)

Article Clinical Neurology

Triheptanoin improves brain energy metabolism in patients with Huntington disease

Isaac Mawusi Adanyeguh et al.

NEUROLOGY (2015)

Article Biotechnology & Applied Microbiology

Riluzole Stimulates BDNF Release from Human Platelets

Patrick Tuerck et al.

BIOMED RESEARCH INTERNATIONAL (2015)

Article Medicine, General & Internal

Huntington disease

Gillian P. Bates et al.

NATURE REVIEWS DISEASE PRIMERS (2015)

Review Neurosciences

Indoleamine 2,3 Dioxygenase as a Potential Therapeutic Target in Huntington's Disease

Gelareh Mazarei et al.

JOURNAL OF HUNTINGTONS DISEASE (2015)

Review Clinical Neurology

An update on Huntington's disease: from the gene to the clinic

Samuel D. Kim et al.

CURRENT OPINION IN NEUROLOGY (2014)

Article Clinical Neurology

Multisource Ascertainment of Huntington Disease in Canada: Prevalence and Population at Risk

Emily R. Fisher et al.

MOVEMENT DISORDERS (2014)

Review Clinical Neurology

Huntington disease: natural history, biomarkers and prospects for therapeutics

Christopher A. Ross et al.

NATURE REVIEWS NEUROLOGY (2014)

Article Multidisciplinary Sciences

SMN2 splicing modifiers improve motor function and longevity in mice with spinal muscular atrophy

Nikolai A. Naryshkin et al.

SCIENCE (2014)

Article Biochemistry & Molecular Biology

Huntingtin promotes mTORC1 signaling in the pathogenesis of Huntington's disease

William M. Pryor et al.

SCIENCE SIGNALING (2014)

Article Clinical Neurology

Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records

Stephen J. W. Evans et al.

JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2013)

Article Clinical Neurology

A Randomized, Double-Blind, Placebo-Controlled Trial of Pridopidine in Huntington's Disease

Karl Kieburtz et al.

MOVEMENT DISORDERS (2013)

Review Clinical Neurology

Epigenetic Mechanisms of Neurodegeneration in Huntington's Disease

Junghee Lee et al.

NEUROTHERAPEUTICS (2013)

Review Biochemistry & Molecular Biology

Huntington's disease: underlying molecular mechanisms and emerging concepts

John Labbadia et al.

TRENDS IN BIOCHEMICAL SCIENCES (2013)

Article Neurosciences

Striatal Volume Contributes to the Prediction of Onset of Huntington Disease in Incident Cases

Elizabeth H. Aylward et al.

BIOLOGICAL PSYCHIATRY (2012)

Article Clinical Neurology

Substance abuse may be a risk factor for earlier onset of Huntington disease

Joanne A. Byars et al.

JOURNAL OF NEUROLOGY (2012)

Article Clinical Neurology

Measures of growth in children at risk for Huntington disease

Jessica K. Lee et al.

NEUROLOGY (2012)

Article Multidisciplinary Sciences

Synthetic zinc finger repressors reduce mutant huntingtin expression in the brain of R6/2 mice

Mireia Garriga-Canut et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2012)

Article Multidisciplinary Sciences

Subunit order of eukaryotic TRiC/CCT chaperonin by cross-linking, mass spectrometry, and combinatorial homology modeling

Nir Kalisman et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2012)

Article Biochemistry & Molecular Biology

Meclizine is neuroprotective in models of Huntington's disease

Vishal M. Gohil et al.

HUMAN MOLECULAR GENETICS (2011)

Review Medicine, Research & Experimental

Oligonucleotide therapeutic approaches for Huntington disease

Dinah W. Y. Sah et al.

JOURNAL OF CLINICAL INVESTIGATION (2011)

Review Health Care Sciences & Services

Management of Huntington's disease: role of tetrabenazine

Marina de Tommaso et al.

THERAPEUTICS AND CLINICAL RISK MANAGEMENT (2011)

Review Pharmacology & Pharmacy

RNA Targeting Therapeutics: Molecular Mechanisms of Antisense Oligonucleotides as a Therapeutic Platform

C. Frank Bennett et al.

ANNUAL REVIEW OF PHARMACOLOGY AND TOXICOLOGY (2010)

Article Clinical Neurology

A Randomized, Placebo-Controlled Trial of Latrepirdine in Huntington Disease

Karl Kieburtz et al.

ARCHIVES OF NEUROLOGY (2010)

Article Neurosciences

Tetrabenazine is neuroprotective in Huntington's disease mice

Hongyu Wang et al.

MOLECULAR NEURODEGENERATION (2010)

Article Clinical Neurology

Sustained Effects of Nonallele-Specific Huntingtin Silencing

Valerie Drouet et al.

ANNALS OF NEUROLOGY (2009)

Article Multidisciplinary Sciences

The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice

Elizabeth A. Thomas et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)

Article Psychiatry

Lamotrigine in motor and mood symptoms of Huntington's disease

Yu-Chih Shen

WORLD JOURNAL OF BIOLOGICAL PSYCHIATRY (2008)

Article Biochemistry & Molecular Biology

Glutamate uptake is reduced in prefrontal cortex in Huntington's disease

Bjornar Hassel et al.

NEUROCHEMICAL RESEARCH (2008)

Article Multidisciplinary Sciences

A small-molecule therapeutic lead for Huntington's disease: Preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse

Vanita Chopra et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2007)

Article Clinical Neurology

Sodium phenylbutyrate in Huntington's disease: A dose-finding study

Penelope Hogarth et al.

MOVEMENT DISORDERS (2007)

Article Clinical Neurology

Riluzole in Huntington's disease: A 3-year, randomized controlled study

G. Bernhard Landwehrmeyer et al.

ANNALS OF NEUROLOGY (2007)

Article Biochemistry & Molecular Biology

Modulation of nucleosome dynamics in Huntington's disease

Edward C. Stack et al.

HUMAN MOLECULAR GENETICS (2007)

Review Neurosciences

Pharmacological properties of BN82451: A novel multitargeting neuroprotective agent

Pierre Etienne Chabrier et al.

CNS DRUG REVIEWS (2007)

Review Neurosciences

Congo red and protein aggregation in neurodegenerative diseases

Petrea Frid et al.

BRAIN RESEARCH REVIEWS (2007)

Article Multidisciplinary Sciences

ESET/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's disease

Hoon Ryu et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2006)

Article Clinical Neurology

Apoptotic cascades as possible targets for inhibiting cell death in Huntington's disease

Lindsay R. Pattison et al.

JOURNAL OF NEUROLOGY (2006)

Article Medicine, Research & Experimental

Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase

M Borrell-Pagès et al.

JOURNAL OF CLINICAL INVESTIGATION (2006)

Article Biochemistry & Molecular Biology

Suppression of polyglutamine-induced toxicity in cell and animal models of Huntington's disease by ubiquilin

H Wang et al.

HUMAN MOLECULAR GENETICS (2006)

Review Pharmacology & Pharmacy

The therapeutic role of creatine in Huntington's disease

H Ryu et al.

PHARMACOLOGY & THERAPEUTICS (2005)

Article Biochemistry & Molecular Biology

Brain-derived neurotrophic factor modulates dopaminergic deficits in a transgenic mouse model of Huntington's disease

JR Pineda et al.

JOURNAL OF NEUROCHEMISTRY (2005)

Article Multidisciplinary Sciences

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

SQ Harper et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)

Article Neurosciences

Amantadine inhibits NMDA receptors by accelerating channel closure during channel block

TA Blanpied et al.

JOURNAL OF NEUROSCIENCE (2005)

Article Biochemistry & Molecular Biology

Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease

G Gardian et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2005)

Article Biochemistry & Molecular Biology

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

M Tanaka et al.

NATURE MEDICINE (2004)

Article Pharmacology & Pharmacy

Neuroprotection in Huntingtons disease:: a 2-year study on minocycline

RM Bonelli et al.

INTERNATIONAL CLINICAL PSYCHOPHARMACOLOGY (2004)

Article Clinical Neurology

Minocycline in Huntington's disease: A pilot study

M Thomas et al.

MOVEMENT DISORDERS (2004)

Article Biochemistry & Molecular Biology

Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease

P Klivenyi et al.

JOURNAL OF NEUROCHEMISTRY (2003)

Article Multidisciplinary Sciences

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

E Hockly et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)

Review Biology

Glutamine and glutamate as vital metabolites

P Newsholme et al.

BRAZILIAN JOURNAL OF MEDICAL AND BIOLOGICAL RESEARCH (2003)

Review Pharmacology & Pharmacy

Lamotrigine - A review of its use in bipolar disorder

DR Goldsmith et al.

Article Multidisciplinary Sciences

Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic model of Huntington's disease

CD Keene et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2002)

Article Biochemistry & Molecular Biology

Neuroprotective effect of eicosapentaenoic acid in hippocampus of rats exposed to γ-irradiation

PE Lonergan et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2002)

Review Biochemistry & Molecular Biology

The biochemistry of n-3 polyunsaturated fatty acids

DB Jump

JOURNAL OF BIOLOGICAL CHEMISTRY (2002)

Article Neurosciences

Amyloid-like inclusions in Huntington's disease

DP McGowan et al.

NEUROSCIENCE (2000)