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Huntington's disease

Nonallele-specific Silencing of Mutant and Wild-type Huntingtin Demonstrates Therapeutic Efficacy in Huntington s Disease Mice

Nonallele-specific Silencing of Mutant and Wild-type Huntingtin Demonstrates Therapeutic Efficacy in Huntington s Disease Mice

... Huntington’s disease (HD) is a fatal neurodegenerative disease caused by mutant huntingtin (htt) protein, and there are currently no effective treatments. Recently, we and others demonstrated that silencing ...

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HUNTINGTON S DISEASE SOCIETY OF AMERICA CENTERS OF EXCELLENCE 2016 Program Description

HUNTINGTON S DISEASE SOCIETY OF AMERICA CENTERS OF EXCELLENCE 2016 Program Description

... Genetic counseling and testing for persons at-risk for Huntington’s disease is a vital part of the mission of the HDSA Center of Excellence program. All Centers of Excellence, regardless of funding level, are ...

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Medications for Huntington s Disease Vicki Wheelock, M.D.

Medications for Huntington s Disease Vicki Wheelock, M.D.

... Occasional stomach upset, dizziness, sleepiness, sexual dysfunction; withdrawal syndrome for Paroxetine... Medications for Mania.[r] ...

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Management of Huntington’s disease: role of tetrabenazine

Management of Huntington’s disease: role of tetrabenazine

... Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder characterized by progressive involuntary movements, neuropsychiatric disturbances, and cognitive ...‘‘huntington’s disease + ...

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Varenicline improves motor and cognitive symptoms in early Huntington’s disease

Varenicline improves motor and cognitive symptoms in early Huntington’s disease

... Huntington’s disease (HD) is a fatal, inherited neurodegenerative disorder characterized by progressive movement characterized by progressive motor, cognitive, and psychi- atric ...

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Overlap between age-at-onset and disease-progression determinants in Huntington disease

Overlap between age-at-onset and disease-progression determinants in Huntington disease

... Unified Huntingtons Disease Rating Scale total functional capacity (A) total motor score (C), a cognitive summary score (E), and body mass index (BMI) (G) increased significantly with a larger ...

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Transgenic animal models for study of the pathogenesis of Huntington’s disease and therapy

Transgenic animal models for study of the pathogenesis of Huntington’s disease and therapy

... Abstract: Huntington’s disease (HD) is caused by a genetic mutation that results in polyglutamine expansion in the N-terminal regions of huntingtin. As a result, this polyQ expansion leads to the misfolding and ...

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Brain structure in juvenile-onset Huntington disease

Brain structure in juvenile-onset Huntington disease

... of disease is defined here as presence of significant motor ...ton’s Disease Rating Scale (UHDRS), a standardized exami- nation performed by a trained ...Huntington’s Disease Network Working Group on ...

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Review of deutetrabenazine: a novel treatment for chorea associated with Huntington's disease

Review of deutetrabenazine: a novel treatment for chorea associated with Huntington's disease

... Abstract: Deutetrabenazine was recently approved for the treatment of chorea in Huntington’s disease (HD) and is the first deuterated medication that has been US Food and Drug Adminis- tration (FDA)-approved for ...

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Bioenergetics in fibroblasts of patients with Huntington disease are associated with age at onset

Bioenergetics in fibroblasts of patients with Huntington disease are associated with age at onset

... of Disease, Frontiers in Cellular Neuroscience, and Cell Death and Disease; is a consultant for the Neurological Institute; and has received research support from Dorpmans-Wigmans ...

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Treatment with a herbal formula B401 enhances neuroprotection and angiogenesis in the R6/2 mouse model of Huntington’s disease

Treatment with a herbal formula B401 enhances neuroprotection and angiogenesis in the R6/2 mouse model of Huntington’s disease

... Abstract: Huntington’s disease (HD) is a neurodegenerative disease characterized by motor dysfunction and early death. Despite years of research, the mechanisms responsible for chronic neurodegeneration of ...

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Neurofilament light protein in blood predicts regional atrophy in Huntington disease

Neurofilament light protein in blood predicts regional atrophy in Huntington disease

... From the Huntingtons Disease Research Centre (E.B.J., L.M.B., S.G., F.B.R., S.J.T., R.I.S., E.J.W.), UCL Institute of Neurology, London, UK; Clinical Neurochemistry Laboratory (K.B., H.Z.), ...

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Clinical manifestations of homozygote allele carriers in Huntington disease

Clinical manifestations of homozygote allele carriers in Huntington disease

... Huntington’s Disease Registry Database, from July 1998 to December 2016, with a larger allele ≥36 CAG repeats within the Huntingtin gene were included in this ...

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Treatment of Huntington's disease Using Medieval Iranian Practice

Treatment of Huntington's disease Using Medieval Iranian Practice

... Huntington’s disease (HD) used by prac- titioners in medieval Persia ...ton’s disease (HD) is an autosomal-dominant disorder characterized by irrepressible motor dysfunction, cogni- tive decline and ...

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Multiple pathways contribute to the pathogenesis of Huntington disease

Multiple pathways contribute to the pathogenesis of Huntington disease

... Huntington's disease (HD) was first described by the American physician George Huntington in 1872. The dis- ease is a genetic disorder of the central nervous system, with symptoms usually consisting of ...

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Clinical diagnosis and management in early Huntington's disease: a review

Clinical diagnosis and management in early Huntington's disease: a review

... Abstract: This review focuses on clinical diagnosis and both pharmacological and nonpharma- cological therapeutic options in early stages of the autosomal dominant inherited neurodegenera- tive Huntington’s ...

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Novel targets for Huntington’s disease: future prospects

Novel targets for Huntington’s disease: future prospects

... few high-quality clinical trials have been conducted, although this is changing with the establishment of such networks as the Huntington’s Study Group and the European Huntington’s Disease Network, who liaise ...

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Quantitative 7T Phase Imaging in Premanifest Huntington Disease

Quantitative 7T Phase Imaging in Premanifest Huntington Disease

... Huntington’s Disease Rating Scale (UHDRS) to determine the total motor score, between 0 and 124, and the diagnostic confidence level, between 0 (healthy, no abnormalities) and 4 (motor abnormalities consistent ...

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Indirect tolerability comparison of Deutetrabenazine and Tetrabenazine for Huntington disease

Indirect tolerability comparison of Deutetrabenazine and Tetrabenazine for Huntington disease

... The European Huntington’s Disease Network (EHDN) REGISTRY study reported that <10% of patients with HD taking medications receive tetrabenazine [18]. Clinical management of chorea is not uniform, and comorbid ...

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HUNTINGTON DISEASE: CURRENT ADVANCES IN PATHOGENESIS AND RECENT THERAPEUTIC STRATEGIES

HUNTINGTON DISEASE: CURRENT ADVANCES IN PATHOGENESIS AND RECENT THERAPEUTIC STRATEGIES

... Huntington's disease (HD) is an inherited autosomal, progressive neurodegenerative disorder associated with involuntary abnormal movements (chorea), cognitive impairments and psychiatric ...

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