相关参考文献
注意:仅列出部分参考文献,下载原文获取全部文献信息。Mutant HTT (huntingtin) impairs mitophagy in a cellular model of Huntington disease
Sandra Franco-Iborra et al.
AUTOPHAGY (2021)
Molecular mechanisms of mitophagy and its roles in neurodegenerative diseases
Xinnan Li et al.
PHARMACOLOGICAL RESEARCH (2021)
Mitophagy impairment in neurodegenerative diseases: Pathogenesis and therapeutic interventions
Shalini Mani et al.
MITOCHONDRION (2021)
Role of mitophagy in mitochondrial quality control: Mechanisms and potential implications for neurodegenerative diseases
Xiao-Le Wang et al.
PHARMACOLOGICAL RESEARCH (2021)
Mitochondrial dysfunction in the development and progression of neurodegenerative diseases
Joseph Johnson et al.
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS (2021)
Mitochondrial fission in Huntington's disease mouse striatum disrupts ER-mitochondria contacts leading to disturbances in Ca2+ efflux and Reactive Oxygen Species (ROS) homeostasis
Marta Cherubini et al.
NEUROBIOLOGY OF DISEASE (2020)
Traffic signaling: new functions of huntingtin and axonal transport in neurological disease
Helene Vitet et al.
CURRENT OPINION IN NEUROBIOLOGY (2020)
Mitochondrial abnormalities in neurodegenerative models and possible interventions: Focus on Alzheimer's disease, Parkinson's disease, Huntington's disease
Patcharapong Pantiya et al.
MITOCHONDRION (2020)
PINK1/PARKIN signalling in neurodegeneration and neuroinflammation
Peter M. J. Quinn et al.
ACTA NEUROPATHOLOGICA COMMUNICATIONS (2020)
Large Animal Models of Huntington's Disease: What We Have Learned and Where We Need to Go Next
David Howland et al.
JOURNAL OF HUNTINGTONS DISEASE (2020)
Regulators of the transsulfuration pathway
Juan I. Sbodio et al.
BRITISH JOURNAL OF PHARMACOLOGY (2019)
Axonal autophagy: Mini-review for autophagy in the CNS
Andrea K. H. Stavoe et al.
NEUROSCIENCE LETTERS (2019)
Drp1/Fis1-mediated mitochondrial fragmentation leads to lysosomal dysfunction in cardiac models of Huntington's disease
A. U. Joshi et al.
JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY (2019)
Ghrelin-mediated improvements in the metabolic phenotype in the R6/2 mouse model of Huntington's disease
Olga Rudenko et al.
JOURNAL OF NEUROENDOCRINOLOGY (2019)
Dynasore Suppresses mTORC1 Activity and Induces Autophagy to Regulate the Clearance of Protein Aggregates in Neurodegenerative Diseases
Yang Chen et al.
NEUROTOXICITY RESEARCH (2019)
Combined cerebral atrophy score in Huntington's disease based on atlas-based MRI volumetry: Sample size calculations for clinical trials
Hans-Peter Mueller et al.
PARKINSONISM & RELATED DISORDERS (2019)
Impaired Redox Signaling in Huntington's Disease: Therapeutic Implications
Bindu D. Paul et al.
FRONTIERS IN MOLECULAR NEUROSCIENCE (2019)
Alterations in synaptic function and plasticity in Huntington disease
Amy I. Smith-Dijak et al.
JOURNAL OF NEUROCHEMISTRY (2019)
Mutant Huntingtin Affects Diabetes and Alzheimer's Markers in Human and Cell Models of Huntington's Disease
Gepoliano Chaves et al.
CELLS (2019)
Dynamics of Dynamin-Related Protein 1 in Alzheimer's Disease and Other Neurodegenerative Diseases
Darryll Oliver et al.
CELLS (2019)
Role of Phosphorylated Tau and Glucose Synthase Kinase 3 Beta in Huntington's Disease Progression
Neha Sawant et al.
JOURNAL OF ALZHEIMERS DISEASE (2019)
DNA Repair Signaling of Huntingtin: The Next Link Between Late-Onset Neurodegenerative Disease and Oxidative DNA Damage
Tamara Maiuri et al.
DNA AND CELL BIOLOGY (2019)
Therapeutic Applications of Cysteamine and Cystamine in Neurodegenerative and Neuropsychiatric Diseases
Bindu D. Paul et al.
FRONTIERS IN NEUROLOGY (2019)
Mitophagy links oxidative stress conditions and neurodegenerative diseases
Ulfuara Shefa et al.
NEURAL REGENERATION RESEARCH (2019)
Modeling Polyglutamine Expansion Diseases with Induced Pluripotent Stem Cells
Swati Naphade et al.
NEUROTHERAPEUTICS (2019)
Huntington's disease: a clinical review
P. McColgan et al.
EUROPEAN JOURNAL OF NEUROLOGY (2018)
A Huntingtin Knockin Pig Model Recapitulates Features of Selective Neurodegeneration in Huntington's Disease
Sen Yan et al.
CELL (2018)
Disrupted striatal neuron inputs and outputs in Huntington's disease
Anton Reiner et al.
CNS NEUROSCIENCE & THERAPEUTICS (2018)
Cause or compensation?Altered neuronal Ca2+ handling in Huntington's disease
James P. Mackay et al.
CNS NEUROSCIENCE & THERAPEUTICS (2018)
Mutant APP and amyloid beta-induced defective autophagy, mitophagy, mitochondrial structural and functional changes and synaptic damage in hippocampal neurons from Alzheimer's disease
P. Hemachandra Reddy et al.
HUMAN MOLECULAR GENETICS (2018)
Human cellular models of medium spiny neuron development and Huntington disease
Monika M. Golas
LIFE SCIENCES (2018)
The adjustment of γ-aminobutyric acidA tonic subunits in Huntington's disease: from transcription to translation to synaptic levels into the neostriatum
Abraham Rosas-Arellano et al.
NEURAL REGENERATION RESEARCH (2018)
Mitophagy in neurodegenerative diseases
Carlo Rodolfo et al.
NEUROCHEMISTRY INTERNATIONAL (2018)
Resveratrol protects neuronal-like cells expressing mutant Huntingtin from dopamine toxicity by rescuing ATG4-mediated autophagosome formation
Chiara Vidoni et al.
NEUROCHEMISTRY INTERNATIONAL (2018)
Increased nuclear DNA damage precedes mitochondrial dysfunction in peripheral blood mononuclear cells from Huntington's disease patients
Georgina Askeland et al.
SCIENTIFIC REPORTS (2018)
Hypothalamic Alterations in Neurodegenerative Diseases and Their Relation to Abnormal Energy Metabolism
Pauline Vercruysse et al.
FRONTIERS IN MOLECULAR NEUROSCIENCE (2018)
Mitochondrial Division Inhibitor 1 (mdivi-1) Protects Neurons against Excitotoxicity through the Modulation of Mitochondrial Function and Intracellular Ca2+ Signaling
Asier Ruiz et al.
FRONTIERS IN MOLECULAR NEUROSCIENCE (2018)
Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease
Sandra Franco-Iborra et al.
FRONTIERS IN NEUROSCIENCE (2018)
Molecular Pathogenesis in Huntington's Disease
S. N. Illarioshkin et al.
BIOCHEMISTRY-MOSCOW (2018)
The cryo-electron microscopy structure of huntingtin
Qiang Guo et al.
NATURE (2018)
The Ubiquitin Receptor ADRM1 Modulates HAP40-Induced Proteasome Activity
Zih-Ning Huang et al.
MOLECULAR NEUROBIOLOGY (2017)
Mdivi-1 ameliorates early brain injury after subarachnoid hemorrhage via the suppression of inflammation-related blood-brain barrier disruption and endoplasmic reticulum stress-based apoptosis
Lin-feng Fan et al.
FREE RADICAL BIOLOGY AND MEDICINE (2017)
Adhesion Regulating Molecule 1 Mediates HAP40 Overexpression-Induced Mitochondrial Defects
Zih-Ning Huang et al.
INTERNATIONAL JOURNAL OF BIOLOGICAL SCIENCES (2017)
Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease
Elisabeth Singer et al.
SCIENTIFIC REPORTS (2017)
Body Weight Is a Robust Predictor of Clinical Progression in Huntington Disease
Jorien M. M. van der Burg et al.
ANNALS OF NEUROLOGY (2017)
Striatal synaptic dysfunction and altered calcium regulation in Huntington disease
Lynn A. Raymond
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2017)
The Putative Drp1 Inhibitor mdivi-1 Is a Reversible Mitochondrial Complex I Inhibitor that Modulates Reactive Oxygen Species
Evan A. Bordt et al.
DEVELOPMENTAL CELL (2017)
Modeling Huntington's disease with patient-derived neurons
Virginia B. Mattis et al.
BRAIN RESEARCH (2017)
The story of George Huntington and his disease
Kalyan B. Bhattacharyya
ANNALS OF INDIAN ACADEMY OF NEUROLOGY (2016)
Human adult neurogenesis across the ages: An immunohistochemical study
C. V. Dennis et al.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2016)
TRiC subunits enhance BDNF axonal transport and rescue striatal atrophy in Huntington's disease
Xiaobei Zhao et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2016)
GSK-3β-induced Tau pathology drives hippocampal neuronal cell death in Huntington's disease: involvement of astrocyte-neuron interactions
F. L'Episcopo et al.
CELL DEATH & DISEASE (2016)
VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease
Xing Guo et al.
NATURE COMMUNICATIONS (2016)
HTT/Huntingtin in selective autophagy and Huntington disease: A foe or a friend within?
Yan-Ning Rui et al.
AUTOPHAGY (2015)
Corticostriatal synaptic adaptations in Huntington's disease
Joshua L. Plotkin et al.
CURRENT OPINION IN NEUROBIOLOGY (2015)
Impaired GAPDH-induced mitophagy contributes to the pathology of Huntington's disease
Sunhee Hwang et al.
EMBO MOLECULAR MEDICINE (2015)
Dopamine and Huntington's disease
Laetitia C. Schwab et al.
EXPERT REVIEW OF NEUROTHERAPEUTICS (2015)
Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics and neuronal damage in Huntington's disease
Maria Manczak et al.
HUMAN MOLECULAR GENETICS (2015)
The Function of Autophagy in Neurodegenerative Diseases
Yoshimitsu Kiriyama et al.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2015)
Fat-free mass and its predictors in Huntington's disease
S. D. Suessmuth et al.
JOURNAL OF NEUROLOGY (2015)
Altered Sensory Experience Exacerbates Stable Dendritic Spine and Synapse Loss in a Mouse Model of Huntington's Disease
Reena Prity Murmu et al.
JOURNAL OF NEUROSCIENCE (2015)
Mitochondrial Quality Control via the PGC1α-TFEB Signaling Pathway Is Compromised by Parkin Q311X Mutation But Independently Restored by Rapamycin
Almas Siddiqui et al.
JOURNAL OF NEUROSCIENCE (2015)
Ectopic expression of the striatal-enriched GTPase Rhes elicits cerebellar degeneration and an ataxia phenotype in Huntington's disease
Supriya Swarnkar et al.
NEUROBIOLOGY OF DISEASE (2015)
PINK1-induced mitophagy promotes neuroprotection in Huntington's disease
B. Khalil et al.
CELL DEATH & DISEASE (2015)
Neural and mesenchymal stem cells in animal models of Huntington's disease: past experiences and future challenges
Irina Kerkis et al.
STEM CELL RESEARCH & THERAPY (2015)
Dopamine and Huntington's disease
Laetitia C. Schwab et al.
EXPERT REVIEW OF NEUROTHERAPEUTICS (2015)
N-Acetylcysteine improves mitochondrial function and ameliorates behavioral deficits in the R6/1 mouse model of Huntington's disease
D. J. Wright et al.
TRANSLATIONAL PSYCHIATRY (2015)
Neonatal iron supplementation potentiates oxidative stress, energetic dysfunction and neurodegeneration in the R6/2 mouse model of Huntington's disease
Kiersten L. Berggren et al.
REDOX BIOLOGY (2015)
Huntington disease
Gillian P. Bates et al.
NATURE REVIEWS DISEASE PRIMERS (2015)
Disruption of astrocyte-neuron cholesterol cross talk affects neuronal function in Huntington's disease
M. Valenza et al.
CELL DEATH AND DIFFERENTIATION (2015)
Mutant huntingtin alters Tau phosphorylation and subcellular distribution
David Blum et al.
HUMAN MOLECULAR GENETICS (2015)
Tau hyperphosphorylation and deregulation of calcineurin in mouse models of Huntington's disease
Maud Gratuze et al.
HUMAN MOLECULAR GENETICS (2015)
Transcription, Epigenetics and Ameliorative Strategies in Huntington's Disease: a Genome-Wide Perspective
Luis M. Valor
MOLECULAR NEUROBIOLOGY (2015)
Huntingtin protein is essential for mitochondrial metabolism, bioenergetics and structure in murine embryonic stem cells
Ismail Ismailoglu et al.
DEVELOPMENTAL BIOLOGY (2014)
The distribution of DNA damage is defined by region-specific susceptibility to DNA damage formation rather than repair differences
Janne M. Strand et al.
DNA REPAIR (2014)
Increased mitochondrial fission and neuronal dysfunction in Huntington's disease: implications for molecular inhibitors of excessive mitochondrial fission
P. Hemachandra Reddy
DRUG DISCOVERY TODAY (2014)
The Regulation of Autophagosome Dynamics by Huntingtin and HAP1 Is Disrupted by Expression of Mutant Huntingtin, Leading to Defective Cargo Degradation
Yvette C. Wong et al.
JOURNAL OF NEUROSCIENCE (2014)
Metabolism in HD: Still a Relevant Mechanism?
Wenzhen Duan et al.
MOVEMENT DISORDERS (2014)
Extrasynaptic NMDA Receptor Involvement in Central Nervous System Disorders
Matthew P. Parsons et al.
NEURON (2014)
Impaired TrkB Receptor Signaling Underlies Corticostriatal Dysfunction in Huntington's Disease
Joshua L. Plotkin et al.
NEURON (2014)
What's wrong with epigenetics in Huntington's disease?
Luis M. Valor et al.
NEUROPHARMACOLOGY (2014)
GluN2A and GluN2B subunit-containing NMDA receptors in hippocampal plasticity
Olivia A. Shipton et al.
PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES (2014)
Potential function for the Huntingtin protein as a scaffold for selective autophagy
Joseph Ochaba et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2014)
Mood disorders in Huntington's disease: from behavior to cellular and molecular mechanisms
Patrick Pla et al.
FRONTIERS IN BEHAVIORAL NEUROSCIENCE (2014)
Huntingtin Regulates Mammary Stem Cell Division and Differentiation
Salah Elias et al.
STEM CELL REPORTS (2014)
Structure, Function, and Pharmacology of NMDA Receptor Channels
V. Vyklicky et al.
PHYSIOLOGICAL RESEARCH (2014)
S-Nitrosylation of Dynamin-Related Protein 1 Mediates Mutant Huntingtin-Induced Mitochondrial Fragmentation and Neuronal Injury in Huntington's Disease
Florian Haun et al.
ANTIOXIDANTS & REDOX SIGNALING (2013)
3-Nitropropionic acid induces autophagy by forming mitochondrial permeability transition pores rather than activatiing the mitochondrial fission pathway
Maria E. Solesio et al.
BRITISH JOURNAL OF PHARMACOLOGY (2013)
Role of oxidative DNA damage in mitochondrial dysfunction and Huntington's disease pathogenesis
Sylvette Ayala-Pena
FREE RADICAL BIOLOGY AND MEDICINE (2013)
PGC-1α, mitochondrial dysfunction, and Huntington's disease
Ashu Johri et al.
FREE RADICAL BIOLOGY AND MEDICINE (2013)
Mitochondrial Diseases of the Brain
Rajnish K. Chaturvedi et al.
FREE RADICAL BIOLOGY AND MEDICINE (2013)
A critical role of astrocyte-mediated nuclear factor-κB-dependent inflammation in Huntington's disease
Han-Yun Hsiao et al.
HUMAN MOLECULAR GENETICS (2013)
Inhibition of mitochondrial fragmentation diminishes Huntington's disease-associated neurodegeneration
Xing Guo et al.
JOURNAL OF CLINICAL INVESTIGATION (2013)
Mutant Huntingtin Alters Retrograde Transport of TrkB Receptors in Striatal Dendrites
Geraldine Liot et al.
JOURNAL OF NEUROSCIENCE (2013)
Tonic mGluR5/CB1-dependent suppression of inhibition as a pathophysiological hallmark in the striatum of mice carrying a mutant form of huntingtin
Anton Dvorzhak et al.
JOURNAL OF PHYSIOLOGY-LONDON (2013)
Three decades of caring for the Venezuelan Huntington's disease families
Nancy S. Wexler
LANCET NEUROLOGY (2013)
Mitochondrial permeability transition pore induces mitochondria injury in Huntington disease
Rodrigo A. Quintanilla et al.
MOLECULAR NEURODEGENERATION (2013)
Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models
Sonia Marco et al.
NATURE MEDICINE (2013)
NMDA receptor subunit diversity: impact on receptor properties, synaptic plasticity and disease
Pierre Paoletti et al.
NATURE REVIEWS NEUROSCIENCE (2013)
Axonal transport deficits and neurodegenerative diseases
Stephanie Millecamps et al.
NATURE REVIEWS NEUROSCIENCE (2013)
VCP Is Essential for Mitochondrial Quality Control by PINK1/Parkin and this Function Is Impaired by VCP Mutations
Nam Chul Kim et al.
NEURON (2013)
The V471A Polymorphism in Autophagy-Related Gene ATG7 Modifies Age at Onset Specifically in Italian Huntington Disease Patients
Silke Metzger et al.
PLOS ONE (2013)
Maintenance of Basal Levels of Autophagy in Huntington's Disease Mouse Models Displaying Metabolic Dysfunction
Barbara Baldo et al.
PLOS ONE (2013)
Targeting H3K4 trimethylation in Huntington disease
Malini Vashishtha et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)
Huntington's disease: underlying molecular mechanisms and emerging concepts
John Labbadia et al.
TRENDS IN BIOCHEMICAL SCIENCES (2013)
A failure in energy metabolism and antioxidant uptake precede symptoms of Huntington's disease in mice
Anibal I. Acuna et al.
NATURE COMMUNICATIONS (2013)
A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases
Kyota Fujita et al.
NATURE COMMUNICATIONS (2013)
Protein oxidation in Huntington disease
M. Alba Sorolla et al.
BIOFACTORS (2012)
Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis
Tamara Ratovitski et al.
CELL CYCLE (2012)
Genetic Correction of Huntington's Disease Phenotypes in Induced Pluripotent Stem Cells
Mahru C. An et al.
CELL STEM CELL (2012)
Mitochondrial DNA damage Is associated with reduced mitochondrial bioenergetics in Huntington's disease
Almas Siddiqui et al.
FREE RADICAL BIOLOGY AND MEDICINE (2012)
Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease
Ulziibat P. Shirendeb et al.
HUMAN MOLECULAR GENETICS (2012)
Pluripotent Stem Cells Models for Huntington's Disease: Prospects and Challenges
Richard L. Carter et al.
JOURNAL OF GENETICS AND GENOMICS (2012)
PGC-1α Negatively Regulates Extrasynaptic NMDAR Activity and Excitotoxicity
Clare Puddifoot et al.
JOURNAL OF NEUROSCIENCE (2012)
The incidence and prevalence of Huntington's disease: A systematic review and meta-analysis
Tamara Pringsheim et al.
MOVEMENT DISORDERS (2012)
Mitigation of augmented extrasynaptic NMDAR signaling and apoptosis in cortico-striatal co-cultures from Huntington's disease mice
Austen J. Milnerwood et al.
NEUROBIOLOGY OF DISEASE (2012)
8OHdG as a marker for Huntington disease progression
Jeffrey D. Long et al.
NEUROBIOLOGY OF DISEASE (2012)
Network Organization of the Huntingtin Proteomic Interactome in Mammalian Brain
Dyna I. Shirasaki et al.
NEURON (2012)
PGC-1α at the intersection of bioenergetics regulation and neuron function: From Huntington's disease to Parkinson's disease and beyond
Taiji Tsunemi et al.
PROGRESS IN NEUROBIOLOGY (2012)
PGC-1α Rescues Huntington's Disease Proteotoxicity by Preventing Oxidative Stress and Promoting TFEB Function
Taiji Tsunemi et al.
SCIENCE TRANSLATIONAL MEDICINE (2012)
Cognitive Deficits in Huntington's Disease: Insights from Animal Models
Elizabeth A. Wang et al.
CURRENT GERIATRICS REPORTS (2012)
Americo Negrette and Huntington's disease
Mariana Moscovich et al.
ARQUIVOS DE NEURO-PSIQUIATRIA (2011)
Huntington's disease, calcium, and mitochondria
Marta Giacomello et al.
BIOFACTORS (2011)
Dynamin-related protein 1 and mitochondrial fragmentation in neurodegenerative diseases
P. Hemachandra Reddy et al.
BRAIN RESEARCH REVIEWS (2011)
Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage
Ulziibat Shirendeb et al.
HUMAN MOLECULAR GENETICS (2011)
Energy deficit in Huntington disease: why it matters
Fanny Mochel et al.
JOURNAL OF CLINICAL INVESTIGATION (2011)
Important Role of Oxidative Stress Biomarkers in Huntington's Disease
Isaac Tunez et al.
JOURNAL OF MEDICINAL CHEMISTRY (2011)
Modifications of p53 and the DNA Damage Response in Cells Expressing Mutant Form of the Protein Huntingtin
Jennifer L. Illuzzi et al.
JOURNAL OF MOLECULAR NEUROSCIENCE (2011)
Mechanisms underlying NMDA receptor synaptic/extrasynaptic distribution and function
Clare M. Gladding et al.
MOLECULAR AND CELLULAR NEUROSCIENCE (2011)
Huntington's disease: from molecular pathogenesis to clinical treatment
Christopher A. Ross et al.
LANCET NEUROLOGY (2011)
PGC-1alpha downstream transcription factors NRF-1 and TFAM are genetic modifiers of Huntington disease
Elahe Taherzadeh-Fard et al.
MOLECULAR NEURODEGENERATION (2011)
Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity
Wenjun Song et al.
NATURE MEDICINE (2011)
Reactive astrocytes and Wnt/β-catenin signaling link nigrostriatal injury to repair in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine model of Parkinson's disease
F. L'Episcopo et al.
NEUROBIOLOGY OF DISEASE (2011)
Huntington's Disease: Can Mice Lead the Way to Treatment?
Zachary R. Crook et al.
NEURON (2011)
Genetic mouse models of Huntington's disease: focus on electrophysiological mechanisms
Carlos Cepeda et al.
ASN NEURO (2010)
Presynaptic dysfunction in Huntington's disease
Jose L. Rozas et al.
BIOCHEMICAL SOCIETY TRANSACTIONS (2010)
Signal transduction to the permeability transition pore
Andrea Rasola et al.
FEBS LETTERS (2010)
Huntingtin facilitates polycomb repressive complex 2
Ihn Sik Seong et al.
HUMAN MOLECULAR GENETICS (2010)
Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease
Jinho Kim et al.
HUMAN MOLECULAR GENETICS (2010)
Mutant huntingtin impairs Ku70-mediated DNA repair
Yasushi Enokido et al.
JOURNAL OF CELL BIOLOGY (2010)
Mutant Huntingtin and Glycogen Synthase Kinase 3-beta Accumulate in Neuronal Lipid Rafts of a Presymptomatic Knock-In Mouse Model of Huntington's Disease
Antonio Valencia et al.
JOURNAL OF NEUROSCIENCE RESEARCH (2010)
Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease
Marta Martinez-Vicente et al.
NATURE NEUROSCIENCE (2010)
Synaptic versus extrasynaptic NMDA receptor signalling: implications for neurodegenerative disorders
Giles E. Hardingham et al.
NATURE REVIEWS NEUROSCIENCE (2010)
Huntingtin Is Required for Mitotic Spindle Orientation and Mammalian Neurogenesis
Juliette D. Godin et al.
NEURON (2010)
Transcriptional dysregulation of coding and non-coding genes in cellular models of Huntington's disease
Angela Bithell et al.
BIOCHEMICAL SOCIETY TRANSACTIONS (2009)
Mitochondrial structural and functional dynamics in Huntington's disease
P. Hemachandra Reddy et al.
BRAIN RESEARCH REVIEWS (2009)
Mouse models of Huntington disease: variations on a theme
Dagmar E. Ehrnhoefer et al.
DISEASE MODELS & MECHANISMS (2009)
Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's disease
Karina Acevedo-Torres et al.
DNA REPAIR (2009)
Impaired PGC-1α function in muscle in Huntington's disease
Rajnish K. Chaturvedi et al.
HUMAN MOLECULAR GENETICS (2009)
Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin
Shu-ichi Okamoto et al.
NATURE MEDICINE (2009)
Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes
Ella Dragileva et al.
NEUROBIOLOGY OF DISEASE (2009)
Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms
Jennifer Bradford et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Rhes, a Striatal Specific Protein, Mediates Mutant-Huntingtin Cytotoxicity
Srinivasa Subramaniam et al.
SCIENCE (2009)
Huntingtin associated protein 1 and its functions
Linda Lin-yan Wu et al.
CELL ADHESION & MIGRATION (2009)
Stoichiometry of Base Excision Repair Proteins Correlates with Increased Somatic CAG Instability in Striatum over Cerebellum in Huntington's Disease Transgenic Mice
Agathi-Vassiliki Goula et al.
PLOS GENETICS (2009)
Evidence of Oxidant Damage in Huntington's Disease: Translational Strategies Using Antioxidants
Edward C. Stack et al.
MITOCHONDRIA AND OXIDATIVE STRESS IN NEURODEGENERATIVE DISORDERS (2008)
Glucose homeostasis in Huntington disease - Abnormalities in insulin sensitivity and early-phase insulin secretion
Nebojsa M. Lalic et al.
ARCHIVES OF NEUROLOGY (2008)
A stress sensitive ER membrane-association domain in Huntingtin protein defines a potential role for Huntingtin in the regulation of autophagy
Randy Singh Atwal et al.
AUTOPHAGY (2008)
Chemical inhibition of the mitochondrial division dynamin reveals its role in Bax/Bak-dependent mitochondrial outer membrane permeabilization
Ann Cassidy-Stone et al.
DEVELOPMENTAL CELL (2008)
Mitochondrial DNA damage and repair in neurodegenerative disorders
Jenq-Lin Yang et al.
DNA REPAIR (2008)
Actin Binding by Hip1 (Huntingtin-interacting Protein 1) and Hip1R (Hip1-related Protein) Is Regulated by Clathrin Light Chain
Jeremy D. Wilbur et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
Huntingtin Modulates Transcription, Occupies Gene Promoters In Vivo, and Binds Directly to DNA in a Polyglutamine-Dependent Manner
Caroline L. Benn et al.
JOURNAL OF NEUROSCIENCE (2008)
Depletion of mitochondrial DNA in leukocytes of patients with poly-Q diseases
Chin-San Liu et al.
JOURNAL OF THE NEUROLOGICAL SCIENCES (2008)
Striatal progenitors derived from human ES cells mature into DARPP32 neurons in vitro and in quinolinic acid-lesioned rats
Laetitia Aubry et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease
Dmitry Lim et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington's disease
Herman B. Fernandes et al.
JOURNAL OF NEUROSCIENCE (2007)
Factors associated with HD CAG repeat instability in Huntington disease
V. C. Wheeler et al.
JOURNAL OF MEDICAL GENETICS (2007)
Huntington's disease and mitochondrial DNA deletions: Event or regular mechanism for mutant Huntingtin protein and CAG repeats expansion?!
Mohammad Mehdi Banoei et al.
CELLULAR AND MOLECULAR NEUROBIOLOGY (2007)
Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity
Randy Singh Atwal et al.
HUMAN MOLECULAR GENETICS (2007)
OGG1 initiates age-dependent CAG trinucleotide expansion in somatic cells
Irina V. Kovtun et al.
NATURE (2007)
Differential morphology and composition of inclusions in the R6/2 mouse and PC12 cell models of Huntington's disease
Jonathan Wanderer et al.
HISTOCHEMISTRY AND CELL BIOLOGY (2007)
Huntingtin interacting proteins are genetic modifiers of neurodegeneration
Linda S. Kaltenbach et al.
PLOS GENETICS (2007)
Oxidative stress and mitochondrial dysfunction in neurodegenerative diseases
E. Trushina et al.
NEUROSCIENCE (2007)
Selective defect of in vivo glycolysis in early Huntington's disease striatum
William J. Powers et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2007)
Wild-type huntingtin participates in protein trafficking between the Golgi and the extracellular space
Anne N. T. Strehlow et al.
HUMAN MOLECULAR GENETICS (2007)
The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis
Erica Rockabrand et al.
HUMAN MOLECULAR GENETICS (2007)
N-methyl-D-aspartate (NMDA) receptor function and excitotoxicity in Huntington's disease
Mannie M. Y. Fan et al.
PROGRESS IN NEUROBIOLOGY (2007)
ESET/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's disease
Hoon Ryu et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2006)
Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells - Functional consequences
Tamara Milakovic et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2006)
Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells:: Effect of histone deacetylase inhibitors
Jorge M. A. Oliveira et al.
JOURNAL OF NEUROSCIENCE (2006)
Cytotoxicity of a mutant huntingtin fragment in yeast involves early alterations in mitochondrial OXPHOS complexes II and III
Asun Solans et al.
HUMAN MOLECULAR GENETICS (2006)
Transcriptional repression of PGC-α by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration
Libin Cui et al.
CELL (2006)
Dynasore, a cell-permeable inhibitor of dynamin
Eric Macia et al.
DEVELOPMENTAL CELL (2006)
Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons
DTW Chang et al.
NEUROBIOLOGY OF DISEASE (2006)
Suppression of polyglutamine-induced toxicity in cell and animal models of Huntington's disease by ubiquilin
H Wang et al.
HUMAN MOLECULAR GENETICS (2006)
Huntingtin-HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington's disease
A Pal et al.
JOURNAL OF CELL BIOLOGY (2006)
Paradigm shift in neuroprotection by NMDA receptor blockade: Memantine and beyond
SA Lipton
NATURE REVIEWS DRUG DISCOVERY (2006)
Reduced expression of the TrkB receptor in Huntington's disease mouse models and in human brain
S Ginés et al.
EUROPEAN JOURNAL OF NEUROSCIENCE (2006)
Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2′dG
SM Hersch et al.
NEUROLOGY (2006)
The chemical biology of clinically tolerated NMDA receptor antagonists
Huei-Sheng Vincent Chen et al.
JOURNAL OF NEUROCHEMISTRY (2006)
Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease
CL Benn et al.
HUMAN MOLECULAR GENETICS (2005)
HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism
IS Seong et al.
HUMAN MOLECULAR GENETICS (2005)
History of genetic disease - The molecular genetics of Huntington disease - a history
GP Bates
NATURE REVIEWS GENETICS (2005)
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease
BI Bae et al.
NEURON (2005)
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease
TS Tang et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)
Potentiation of NMDA receptor-mediated excitotoxicity linked with intrinsic apoptotic pathway in YAC transgenic mouse model of Huntington's disease
MM Zeron et al.
MOLECULAR AND CELLULAR NEUROSCIENCE (2004)
Huntingtin-protein interactions and the pathogenesis of Huntington's disease|
SH Li et al.
TRENDS IN GENETICS (2004)
Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro
E Trushina et al.
MOLECULAR AND CELLULAR BIOLOGY (2004)
Huntingtin-interacting protein HIP14 is a palmitoyl transferase involved in palmitoylation and trafficking of multiple neuronal proteins
K Huang et al.
NEURON (2004)
Americo Negrette (1924 to 2003) - Diagnosing Huntington disease in Venezuela
MS Okun et al.
NEUROLOGY (2004)
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release
YS Choo et al.
HUMAN MOLECULAR GENETICS (2004)
Genetic mouse models of Huntington's and Parkinson's diseases: illuminating but imperfect
MS Levine et al.
TRENDS IN NEUROSCIENCES (2004)
Circulating and cerebrospinal fluid ghrelin and leptin: potential role in altered body weight in Huntington's disease
V Popovic et al.
EUROPEAN JOURNAL OF ENDOCRINOLOGY (2004)
Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats
LB Menalled et al.
JOURNAL OF COMPARATIVE NEUROLOGY (2003)
Minocycline inhibits caspase-independent and -dependent mitochondrial cell death pathways in models of Huntington's disease
X Wang et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes
C Zuccato et al.
NATURE GENETICS (2003)
Nuclease-deficient FEN-1 blocks rad51/BRCA1-mediated repair and causes trinucleotide repeat instability
C Spiro et al.
MOLECULAR AND CELLULAR BIOLOGY (2003)
Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release
H Li et al.
HUMAN MOLECULAR GENETICS (2003)
Huntingtin and Huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1
TS Tang et al.
NEURON (2003)
Overexpression and nuclear accumulation of glyceraldehyde-3-phosphate dehydrogenase in a transgenic mouse model of Huntington's disease
VV Senatorov et al.
MOLECULAR AND CELLULAR NEUROSCIENCE (2003)
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines
AV Panov et al.
NATURE NEUROSCIENCE (2002)
Lessons from animal models of Huntington's disease
DC Rubinsztein
TRENDS IN GENETICS (2002)
Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease
MM Zeron et al.
NEURON (2002)
Mouse models of Huntington's disease
LB Menalled et al.
TRENDS IN PHARMACOLOGICAL SCIENCES (2002)
Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease
MB Bogdanov et al.
JOURNAL OF NEUROCHEMISTRY (2001)
Electrophysiological and morphological changes in striatal spiny neurons in R6/2 Huntington's disease transgenic mice
GJ Klapstein et al.
JOURNAL OF NEUROPHYSIOLOGY (2001)
NMDA receptor function in mouse models of Huntington disease
C Cepeda et al.
JOURNAL OF NEUROSCIENCE RESEARCH (2001)
Expression of the Huntington's disease transgene in neural stem cell cultures from R6/2 transgenic mice
Q Chu-LaGraff et al.
BRAIN RESEARCH BULLETIN (2001)
Polyglutamine-expanded Huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95
Y Sun et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
Comparative analysis of superoxide dismutase activity between acute pharmacological models and a transgenic mouse model of Huntington's disease
A Santamaría et al.
NEUROCHEMICAL RESEARCH (2001)
Trinucleotide expansion in haploid germ cells by gap repair
IV Kovtun et al.
NATURE GENETICS (2001)
Progression of symptoms in the early and middle stages of Huntington disease
SC Kirkwood et al.
ARCHIVES OF NEUROLOGY (2001)
Dominant phenotypes produced by the HD mutation in STHdhQ111 striatal cells
F Trettel et al.
HUMAN MOLECULAR GENETICS (2000)
Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy
KB Kegel et al.
JOURNAL OF NEUROSCIENCE (2000)
The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
JS Steffan et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2000)
Wild-type Huntingtin protects from apoptosis upstream of caspase-3
D Rigamonti et al.
JOURNAL OF NEUROSCIENCE (2000)
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
A Yamamoto et al.
CELL (2000)