Mutant huntingtin oligomers drive early human pathogenesis in Huntington’s disease (original) (raw)

Soluble mutant huntingtin drives early human pathogenesis in Huntington’s disease

Cellular and Molecular Life Sciences

Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease

Solen Gokhan

Proceedings of the National Academy of Sciences of the United States of America, 2016

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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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Mono-and Biallelic Inactivation of Huntingtin Gene in Patient-Specific Induced Pluripotent Stem Cells Reveal HTT Roles in Striatal Development and Neuronal Functions

Nicole Déglon

J Huntingtons Dis, 2024

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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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In vivoprogressive degeneration of Huntington’s disease patient-derived neurons reveals human-specific pathological phenotypes

nick allen

2020

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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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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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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 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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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

Solen Gokhan

The Journal of Neuroscience

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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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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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Benefits of global mutant huntingtin lowering diminish over time in a Huntington’s disease mouse model

Deanna Marchionini

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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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Analysis of mutant and total huntingtin expression in Huntington’s disease murine models

Douglas Macdonald

Scientific Reports, 2020

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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 Is Cleared from the Brain via Active Mechanisms in Huntington Disease

Ji-joon Song

The Journal of Neuroscience, 2020

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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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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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Reversal of cellular phenotypes in neural cells derived from Huntington's disease monkey-induced pluripotent stem cells

Rangsun Parnpai

Stem cell reports, 2014

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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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Recent advances in understanding the pathogenesis of Huntington's disease

Danilo Tagle

Trends in Neurosciences, 1999

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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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A Fresh Look at Huntingtin mRNA Processing in Huntington’s Disease

Abu Lais

Journal of Huntington's Disease, 2018

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Impairment of developmental stem cell-mediated striatal neurogenesis and pluripotency genes in a knock-in model of Huntington's disease

Solen Gokhan

Proceedings of the National Academy of Sciences of the United States of America, 2009

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Human Huntington's Disease iPSC-Derived Cortical Neurons Display Altered Transcriptomics, Morphology, and Maturation

Virginia Mattis

Cell reports, 2018

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Early transcriptional changes linked to naturally occurring Huntington's disease mutations in neural derivatives of human embryonic stem cells

Nicole Déglon

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HUNTINGTON'S DISEASE: UNDERSTANDING THE PATHOPHYSIOLOGY THROUGH THE HUNTINGTIN GENE

Editor iajps

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Wild-Type Huntingtin Plays a Role in Brain Development and Neuronal Survival

Anton Reiner

Molecular Neurobiology, 2003

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Mutant huntingtin oligomers drive early human pathogenesis in Huntington’s disease (original) (raw)

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