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The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients

Scientific reports, 2017-05, Vol.7 (1), p.1307-10, Article 1307

2017

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Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice

Nature neuroscience, 2016-04, Vol.19 (4), p.623

2016

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Mind the gap: Models in multiple species needed for therapeutic development in Huntington's disease

Movement disorders, 2014-09, Vol.29 (11), p.1397-1403

2014

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Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease

Proceedings of the National Academy of Sciences - PNAS, 2013-02, Vol.110 (6), p.2366-2370

2013

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Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175

PloS one, 2012-12, Vol.7 (12), p.e49838-e49838

2012

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Promotion of somatic CAG repeat expansion by Fan1 knock-out in Huntington's disease knock-in mice is blocked by Mlh1 knock-out

Human molecular genetics, 2020-11, Vol.29 (18), p.3044-3053

2020

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Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington's disease

PloS one, 2012-12, Vol.7 (12), p.e50717-e50717

2012

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MicroRNA signatures of endogenous Huntingtin CAG repeat expansion in mice

PloS one, 2018-01, Vol.13 (1), p.e0190550-e0190550

2018

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Focal Loss of the Glutamate Transporter EAAT2 in a Transgenic Rat Model of SOD1 Mutant-Mediated Amyotrophic Lateral Sclerosis (ALS)

Proceedings of the National Academy of Sciences - PNAS, 2002-02, Vol.99 (3), p.1604-1609

2002

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Disruption of Dynein/Dynactin Inhibits Axonal Transport in Motor Neurons Causing Late-Onset Progressive Degeneration

Neuron (Cambridge, Mass.), 2002-05, Vol.34 (5), p.715-727

2002

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Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

Neurobiology of disease, 2009-09, Vol.35 (3), p.319-336

2009

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Large genetic animal models of Huntington's Disease

Journal of Huntington's disease, 2013, Vol.2 (1), p.3

2013

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Genetic knock-down of HDAC3 does not modify disease-related phenotypes in a mouse model of Huntington's disease

PloS one, 2012-02, Vol.7 (2), p.e31080

2012

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Time-Restricted Feeding Improves Circadian Dysfunction as well as Motor Symptoms in the Q175 Mouse Model of Huntington's Disease

eNeuro, 2018-01, Vol.5 (1), p.ENEURO.0431-17.2017

2018

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A8 Large animal models in huntington’s disease: conceptual overview: what model to use for what purpose

Journal of neurology, neurosurgery and psychiatry, 2016-09, Vol.87 (Suppl 1), p.A3-A3

Howland, David

2016

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Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease mice

Scientific reports, 2017-10, Vol.7 (1), p.14275-14, Article 14275

2017

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Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice

Human molecular genetics, 2012-05, Vol.21 (9), p.1954-1967

2012

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Impaired Spinal Cord Glutamate Transport Capacity and Reduced Sensitivity to Riluzole in a Transgenic Superoxide Dismutase Mutant Rat Model of Amyotrophic Lateral Sclerosis

The Journal of neuroscience, 2003-03, Vol.23 (5), p.1688-1696

2003

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Brain Region Specific Actions of Regulator of G Protein Signaling 4 Oppose Morphine Reward and Dependence but Promote Analgesia

Biological psychiatry (1969), 2010-04, Vol.67 (8), p.761-769

2010

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A transgenic minipig model of Huntington's Disease

Journal of Huntington's disease, 2013, Vol.2 (1), p.47

2013

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Mitochondrial Metabolism in a Large-Animal Model of Huntington Disease: The Hunt for Biomarkers in the Spermatozoa of Presymptomatic Minipigs

Neuro-degenerative diseases, 2017-01, Vol.17 (4-5), p.213-226

2017

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A transgenic rat model of Alzheimer's disease with extracellular Aβ deposition

Neurobiology of aging, 2009-07, Vol.30 (7), p.1078-1090

2009

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Comprehensive behavioral testing in the R6/2 mouse model of Huntington's disease shows no benefit from CoQ10 or minocycline

PloS one, 2010-03, Vol.5 (3), p.e9793-e9793

2010

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Mutated Huntingtin Causes Testicular Pathology in Transgenic Minipig Boars

Neuro-degenerative diseases, 2016-01, Vol.16 (3-4), p.245-259

2016

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The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients

Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice

Mind the gap: Models in multiple species needed for therapeutic development in Huntington's disease

Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease

Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175

Promotion of somatic CAG repeat expansion by Fan1 knock-out in Huntington's disease knock-in mice is blocked by Mlh1 knock-out

Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington's disease

MicroRNA signatures of endogenous Huntingtin CAG repeat expansion in mice

Focal Loss of the Glutamate Transporter EAAT2 in a Transgenic Rat Model of SOD1 Mutant-Mediated Amyotrophic Lateral Sclerosis (ALS)

Disruption of Dynein/Dynactin Inhibits Axonal Transport in Motor Neurons Causing Late-Onset Progressive Degeneration

Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

Large genetic animal models of Huntington's Disease

Genetic knock-down of HDAC3 does not modify disease-related phenotypes in a mouse model of Huntington's disease

Time-Restricted Feeding Improves Circadian Dysfunction as well as Motor Symptoms in the Q175 Mouse Model of Huntington's Disease

A8 Large animal models in huntington’s disease: conceptual overview: what model to use for what purpose

Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease mice

Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice

Impaired Spinal Cord Glutamate Transport Capacity and Reduced Sensitivity to Riluzole in a Transgenic Superoxide Dismutase Mutant Rat Model of Amyotrophic Lateral Sclerosis

Brain Region Specific Actions of Regulator of G Protein Signaling 4 Oppose Morphine Reward and Dependence but Promote Analgesia

A transgenic minipig model of Huntington's Disease

Mitochondrial Metabolism in a Large-Animal Model of Huntington Disease: The Hunt for Biomarkers in the Spermatozoa of Presymptomatic Minipigs

A transgenic rat model of Alzheimer's disease with extracellular Aβ deposition

Genetic deletion of transglutaminase 2 does not rescue the phenotypic deficits observed in R6/2 and zQ175 mouse models of Huntington's disease

Comprehensive behavioral testing in the R6/2 mouse model of Huntington's disease shows no benefit from CoQ10 or minocycline

Mutated Huntingtin Causes Testicular Pathology in Transgenic Minipig Boars

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