Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease (original) (raw)
Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease
Michelle Gray
Nature Medicine, 2014
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Mutant Huntingtin in Glial Cells Exacerbates Neurological Symptoms of Huntington Disease Mice
Shihua Li
Journal of Biological Chemistry, 2010
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Benefits of global mutant huntingtin lowering diminish over time in a Huntington’s disease mouse model
Deanna Marchionini
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Postnatal and adult consequences of loss of huntingtin during development: Implications for Huntington's disease
Solen Gokhan
Neurobiology of Disease, 2016
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Mutant huntingtin oligomers drive early human pathogenesis in Huntington’s disease
nick allen
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Analysis of mutant and total huntingtin expression in Huntington’s disease murine models
Douglas Macdonald
Scientific Reports, 2020
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Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease
Rona Graham
Human Molecular Genetics, 2008
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Soluble mutant huntingtin drives early human pathogenesis in Huntington’s disease
nick allen
Cellular and Molecular Life Sciences
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Cortical Efferents Lacking Mutant huntingtin Improve Striatal Neuronal Activity and Behavior in a Conditional Mouse Model of Huntington's Disease
George Rebec
The Journal of neuroscience : the official journal of the Society for Neuroscience, 2015
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Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington's Disease Knock-In Mouse Models
Manuela Basso
Journal of Huntington's disease, 2018
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Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage
Lesley Jones
Human Molecular Genetics, 2007
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Mutant huntingtin reduction in astrocytes slows disease progression in the bachd conditional huntington's disease mouse model
Joshua Barry
Human molecular genetics, 2018
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Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease
Ruth Luthi-Carter
Human Molecular Genetics, 2002
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A YAC Mouse Model for Huntington’s Disease with Full-Length Mutant Huntingtin, Cytoplasmic Toxicity, and Selective Striatal Neurodegeneration
Anthony Phillips
Neuron, 1999
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Full-Length Human Mutant Huntingtin with a Stable Polyglutamine Repeat Can Elicit Progressive and Selective Neuropathogenesis in BACHD Mice
Michelle Gray
Journal of Neuroscience, 2008
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HUNTINGTON'S DISEASE: UNDERSTANDING THE PATHOPHYSIOLOGY THROUGH THE HUNTINGTIN GENE
Editor iajps
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Loss of normal huntingtin function: new developments in Huntington's disease research
Ferdinando Squitieri
Trends in Neurosciences, 2001
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Loss-of-Huntingtin in Medial and Lateral Ganglionic Lineages Differentially Disrupts Regional Interneuron and Projection Neuron Subtypes and Promotes Huntington's Disease-Associated Behavioral, Cellular, and Pathological Hallmarks
Aldrin Molero
The Journal of Neuroscience, 2019
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Cellular Localization of Huntingtin in Striatal and Cortical Neurons in Rats: Lack of Correlation with Neuronal Vulnerability in Huntington's Disease
Francesca Fusco
1999
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Mutant Huntingtin Is Cleared from the Brain via Active Mechanisms in Huntington Disease
Ji-joon Song
The Journal of Neuroscience, 2020
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Molecular Pathophysiological Mechanisms in Huntington’s Disease
Anamaria Jurcau
Biomedicines
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In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects
N. Dufour, Reid Gilmore, Philippe Hantraye, Nicole Déglon
Human Molecular Genetics, 2010
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Recent advances in understanding the pathogenesis of Huntington's disease
Danilo Tagle
Trends in Neurosciences, 1999
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Lack of mutant huntingtin in cortical efferents improves behavioral inflexibility and corticostriatal dynamics in Huntington's disease mice
George Rebec
Journal of Neurophysiology, 2019
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Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice
vicky gonzales
Human Molecular Genetics, 2004
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Subcellular localisation and formation of huntingtin aggregates correlates with symptom onset and progression in a Huntington’s disease model
Marie Bondulich
Brain Communications, 2020
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Decreased expression of striatal signaling genes in a mouse model of Huntington's disease
Ruth Luthi-Carter
Human Molecular Genetics, 2000
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Huntington's Disease: From Mutant Huntingtin Protein to Neurotrophic Factor Therapy
Youssef Sari
International Journal
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Subcellular Localization And Formation Of Huntingtin Aggregates Correlates With Symptom Onset And Progression In A Huntington’S Disease Model
Michael Flower
Brain Communications, 2020
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Recent Advances on the Pathogenesis of Huntington's Disease
Patrik Brundin
Experimental Neurology, 1999
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