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Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

Nature medicine, 2011-03, Vol.17 (3), p.377-382

2011

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A thiol probe for measuring unfolded protein load and proteostasis in cells

Nature communications, 2017-09, Vol.8 (1), p.474-11, Article 474

2017

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Modeling Doxorubicin-Induced Cardiotoxicity in Human Pluripotent Stem Cell Derived-Cardiomyocytes

Scientific reports, 2016-05, Vol.6 (1), p.25333, Article 25333

2016

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Early Increase in Extrasynaptic NMDA Receptor Signaling and Expression Contributes to Phenotype Onset in Huntington's Disease Mice

Neuron (Cambridge, Mass.), 2010-01, Vol.65 (2), p.178-190

2010

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Reversal of Phenotypic Abnormalities by CRISPR/Cas9-Mediated Gene Correction in Huntington Disease Patient-Derived Induced Pluripotent Stem Cells

Stem cell reports, 2017-03, Vol.8 (3), p.619-633

2017

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Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin

Nature medicine, 2009-12, Vol.15 (12), p.1407-1413

2009

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A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease

The Journal of experimental medicine, 2008-08, Vol.205 (8), p.1869-1877

2008

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Generation of three isogenic gene-edited Huntington’s disease human embryonic stem cell lines with DOX-inducible NGN2 expression cassette in the AAVS1 safe locus

Stem cell research, 2024-06, Vol.77, p.103408, Article 103408

2024

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Manipulation of microbiota reveals altered callosal myelination and white matter plasticity in a model of Huntington disease

Neurobiology of disease, 2019-07, Vol.127, p.65-75

2019

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Claudin-11 in health and disease: implications for myelin disorders, hearing, and fertility

Frontiers in cellular neuroscience, 2024-01, Vol.17, p.1344090

2024

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Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease

Neurobiology of disease, 2015-04, Vol.76, p.46-56

2015

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Rescue of aberrant huntingtin palmitoylation ameliorates mutant huntingtin-induced toxicity

Neurobiology of disease, 2021-10, Vol.158, p.105479-105479, Article 105479

2021

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CHCHD2 up-regulation in Huntington disease mediates a compensatory protective response against oxidative stress

Cell death & disease, 2024-02, Vol.15 (2), p.126-126, Article 126

2024

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Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes

The Journal of neuroscience, 2010-08, Vol.30 (32), p.10844-10850

2010

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Narciclasine attenuates diet-induced obesity by promoting oxidative metabolism in skeletal muscle

PLoS biology, 2017-02, Vol.15 (2), p.e1002597-e1002597

2017

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A homozygous loss-of-function CAMK2A mutation causes growth delay, frequent seizures and severe intellectual disability

eLife, 2018-05, Vol.7

2018

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Pluripotent stem cell-derived models of neurological diseases reveal early transcriptional heterogeneity

Genome Biology, 2021-03, Vol.22 (1), p.73-73, Article 73

2021

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Oligodendroglia and myelin pathology in fragile X syndrome

Journal of neurochemistry, 2024-09, Vol.168 (9), p.2214-2226

2024

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Elevated de novo protein synthesis in FMRP-deficient human neurons and its correction by metformin treatment

Molecular autism, 2020-05, Vol.11 (1), p.41-41, Article 41

2020

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Altered adult hippocampal neurogenesis in the YAC128 transgenic mouse model of Huntington disease

Neurobiology of disease, 2011-02, Vol.41 (2), p.249-260

2011

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Reprint of: Manipulation of microbiota reveals altered callosal myelination and white matter plasticity in a model of Huntington disease

Neurobiology of disease, 2020-02, Vol.135, p.104744-104744, Article 104744

2020

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Generation of a human induced pluripotent stem cell line from a patient with hypomyelinating leukodystrophy 22 (HLD22)

Stem cell research, 2023-09, Vol.71, p.103174-103174, Article 103174

2023

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Choosing an animal model for the study of Huntington's disease

Nature reviews. Neuroscience, 2013-10, Vol.14 (10), p.708-721

2013

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Partial rescue of some features of Huntington Disease in the genetic absence of caspase-6 in YAC128 mice

Neurobiology of disease, 2015-04, Vol.76, p.24-36

2015

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Histone modifications and p53 binding poise the p21 promoter for activation in human embryonic stem cells

Scientific reports, 2016-06, Vol.6 (1), p.28112-28112, Article 28112

2016

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Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

A thiol probe for measuring unfolded protein load and proteostasis in cells

Modeling Doxorubicin-Induced Cardiotoxicity in Human Pluripotent Stem Cell Derived-Cardiomyocytes

Early Increase in Extrasynaptic NMDA Receptor Signaling and Expression Contributes to Phenotype Onset in Huntington's Disease Mice

Reversal of Phenotypic Abnormalities by CRISPR/Cas9-Mediated Gene Correction in Huntington Disease Patient-Derived Induced Pluripotent Stem Cells

Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin

A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease

Generation of three isogenic gene-edited Huntington’s disease human embryonic stem cell lines with DOX-inducible NGN2 expression cassette in the AAVS1 safe locus

Manipulation of microbiota reveals altered callosal myelination and white matter plasticity in a model of Huntington disease

Claudin-11 in health and disease: implications for myelin disorders, hearing, and fertility

Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease

Rescue of aberrant huntingtin palmitoylation ameliorates mutant huntingtin-induced toxicity

CHCHD2 up-regulation in Huntington disease mediates a compensatory protective response against oxidative stress

Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes

Narciclasine attenuates diet-induced obesity by promoting oxidative metabolism in skeletal muscle

A homozygous loss-of-function CAMK2A mutation causes growth delay, frequent seizures and severe intellectual disability

Pluripotent stem cell-derived models of neurological diseases reveal early transcriptional heterogeneity

Oligodendroglia and myelin pathology in fragile X syndrome

Elevated de novo protein synthesis in FMRP-deficient human neurons and its correction by metformin treatment

Altered adult hippocampal neurogenesis in the YAC128 transgenic mouse model of Huntington disease

Reprint of: Manipulation of microbiota reveals altered callosal myelination and white matter plasticity in a model of Huntington disease

Generation of a human induced pluripotent stem cell line from a patient with hypomyelinating leukodystrophy 22 (HLD22)

Choosing an animal model for the study of Huntington's disease

Partial rescue of some features of Huntington Disease in the genetic absence of caspase-6 in YAC128 mice

Histone modifications and p53 binding poise the p21 promoter for activation in human embryonic stem cells

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