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

Progressive sleep and electroencephalogram changes in mice carrying the Huntington’s disease mutation

(2013) Sandor Kantor et al.   BRAIN

Longitudinal analysis of the electroencephalogram and sleep phenotype in the R6/2 mouse model of Huntington’s disease

(2013) Simon P. Fisher et al.   BRAIN

Hypothalamic expression of mutant huntingtin contributes to the development of depressive-like behavior in the BAC transgenic mouse model of Huntington's disease

(2013) Sofia Hult Lundh et al.   HUMAN MOLECULAR GENETICS

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

(2013) K. Sathasivam et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Motor, emotional and cognitive deficits in adult BACHD mice: A model for Huntington's disease

(2012) Yah-se K. Abada et al.   BEHAVIOURAL BRAIN RESEARCH

Striatal atrophy and dendritic alterations in a knock-in mouse model of Huntington's disease

(2012) Renata P. Lerner et al.   BRAIN RESEARCH BULLETIN

Significantly differential diffusion of neuropathological aggregates in the brain of transgenic mice carrying N-terminal mutant huntingtin fused with green fluorescent protein

(2012) Pei-Hsun Cheng et al.   Brain Structure & Function

Purkinje cell dysfunction and loss in a knock-in mouse model of Huntington Disease

(2012) S.E. Dougherty et al.   EXPERIMENTAL NEUROLOGY

Circadian and sleep disorder in Huntington's disease

(2012) A. Jennifer Morton   EXPERIMENTAL NEUROLOGY

Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice

(2012) M. A. Pouladi et al.   HUMAN MOLECULAR GENETICS

A fully humanized transgenic mouse model of Huntington disease

(2012) Amber L. Southwell et al.   HUMAN MOLECULAR GENETICS

Mutant huntingtin impairs immune cell migration in Huntington disease

(2012) Wanda Kwan et al.   JOURNAL OF CLINICAL INVESTIGATION

A Novel BACHD Transgenic Rat Exhibits Characteristic Neuropathological Features of Huntington Disease

(2012) L. Yu-Taeger et al.   JOURNAL OF NEUROSCIENCE

Complex reorganization and predominant non-homologous repair following chromosomal breakage in karyotypically balanced germline rearrangements and transgenic integration

(2012) Colby Chiang et al.   NATURE GENETICS

“Ectopic” theta oscillations and interictal activity during slow-wave state in the R6/1 mouse model of Huntington's disease

(2012) Michele Pignatelli et al.   NEUROBIOLOGY OF DISEASE

Spatiotemporal mapping of brain atrophy in mouse models of Huntington's disease using longitudinal in vivo magnetic resonance imaging

(2012) Manisha Aggarwal et al.   NEUROIMAGE

Comprehensive Behavioral and Molecular Characterization of a New Knock-In Mouse Model of Huntington’s Disease: zQ175

(2012) Liliana B. Menalled et al.   PLoS One

PGC-1  Rescues Huntington's Disease Proteotoxicity by Preventing Oxidative Stress and Promoting TFEB Function

(2012) T. Tsunemi et al.   Science Translational Medicine

Characterizing cognitive aging of working memory and executive function in animal models

(2012) Jennifer L. Bizon et al.   Frontiers in Aging Neuroscience

Molecular characterization of skeletal muscle atrophy in the R6/2 mouse model of Huntington's disease

(2011) Pengxiang She et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Longitudinal analyses of operant performance on the serial implicit learning task (SILT) in the YAC128 Huntington's disease mouse line

(2011) Simon P. Brooks et al.   BRAIN RESEARCH BULLETIN

Selective cognitive impairment in the YAC128 Huntington's disease mouse

(2011) Simon P. Brooks et al.   BRAIN RESEARCH BULLETIN

Light and electron microscopic characterization of the evolution of cellular pathology in the R6/1 Huntington's disease transgenic mice

(2011) Zubeyde Bayram-Weston et al.   BRAIN RESEARCH BULLETIN

Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington's disease transgenic mice

(2011) Simon P. Brooks et al.   BRAIN RESEARCH BULLETIN

Cognitive Impairment in Huntington Disease: Diagnosis and Treatment

(2011) Jane S. Paulsen   Current Neurology and Neuroscience Reports

Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment

(2011) Andrew T.N. Tebbenkamp et al.   HUMAN MOLECULAR GENETICS

Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative

(2011) Andrew T.N. Tebbenkamp et al.   HUMAN MOLECULAR GENETICS

Long-term memory deficits in Huntington's disease are associated with reduced CBP histone acetylase activity

(2011) A. Giralt et al.   HUMAN MOLECULAR GENETICS

Increased PKA signaling disrupts recognition memory and spatial memory: role in Huntington's disease

(2011) Albert Giralt et al.   HUMAN MOLECULAR GENETICS

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

(2011) Wenjun Song et al.   NATURE MEDICINE

Natural history of disease in the YAC128 mouse reveals a discrete signature of pathology in Huntington disease

(2011) Jeffrey B. Carroll et al.   NEUROBIOLOGY OF DISEASE

Structural MRI detects progressive regional brain atrophy and neuroprotective effects in N171-82Q Huntington's disease mouse model

(2011) Yong Cheng et al.   NEUROIMAGE

Combined Treatment with the Mood Stabilizers Lithium and Valproate Produces Multiple Beneficial Effects in Transgenic Mouse Models of Huntington's Disease

(2011) Chi-Tso Chiu et al.   NEUROPSYCHOPHARMACOLOGY

Huntington's disease knock-in male mice show specific anxiety-like behaviour and altered neuronal maturation

(2011) Sophie Orvoen et al.   NEUROSCIENCE LETTERS

Executive Decision-Making in the Domestic Sheep

(2011) A. Jennifer Morton et al.   PLoS One

Sex-Dependent Changes in Social Behaviors in Motor Pre-Symptomatic R6/1 Mice

(2011) Susanna Pietropaolo et al.   PLoS One

Sexually Dimorphic Serotonergic Dysfunction in a Mouse Model of Huntington's Disease and Depression

(2011) Thibault Renoir et al.   PLoS One

Changes in striatal procedural memory coding correlate with learning deficits in a mouse model of Huntington disease

(2011) S. Cayzac et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Caspase-6 and neurodegeneration

(2011) Rona K. Graham et al.   TRENDS IN NEUROSCIENCES

Asymptomatic Sleep Abnormalities Are a Common Early Feature in Patients with Huntington’s Disease

(2010) Anna O. G. Goodman et al.   Current Neurology and Neuroscience Reports

Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease

(2010) Takashi Kudo et al.   EXPERIMENTAL NEUROLOGY

Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression

(2010) M. A. Pouladi et al.   HUMAN MOLECULAR GENETICS

Early autophagic response in a novel knock-in model of Huntington disease

(2010) Mary Y. Heng et al.   HUMAN MOLECULAR GENETICS

Expression of Huntington's disease protein results in apoptotic neurons in the brains of cloned transgenic pigs

(2010) Dongshan Yang et al.   HUMAN MOLECULAR GENETICS

An ovine transgenic Huntington's disease model

(2010) J. C. Jacobsen et al.   HUMAN MOLECULAR GENETICS

Cerebellar lipid differences between R6/1 transgenic mice and humans with Huntington’s disease

(2010) Christine A. Denny et al.   JOURNAL OF NEUROCHEMISTRY

Huntington's disease: from molecular pathogenesis to clinical treatment

(2010) Christopher A Ross et al.   LANCET NEUROLOGY

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

(2010) Jessica M. Simpson et al.   NEUROBIOLOGY OF DISEASE

Before chorea: Pre-Huntington mild cognitive impairment

(2010) D. G. Lichter et al.   NEUROLOGY

Matrix Metalloproteinases Are Modifiers of Huntingtin Proteolysis and Toxicity in Huntington's Disease

(2010) John P. Miller et al.   NEURON

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

(2010) Austen J. Milnerwood et al.   NEURON

Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin

(2009) M. A. Pouladi et al.   BRAIN

Mouse models of Huntington disease: variations on a theme

(2009) D. E. Ehrnhoefer et al.   Disease Models & Mechanisms

Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models

(2009) Jack Phan et al.   HUMAN MOLECULAR GENETICS

Intrabody Gene Therapy Ameliorates Motor, Cognitive, and Neuropathological Symptoms in Multiple Mouse Models of Huntington's Disease

(2009) A. L. Southwell et al.   JOURNAL OF NEUROSCIENCE

Beyond the brain: widespread pathology in Huntington's disease

(2009) Jorien MM van der Burg et al.   LANCET NEUROLOGY

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

(2009) Shu-ichi Okamoto et al.   NATURE MEDICINE

Tests to assess motor phenotype in mice: a user's guide

(2009) Simon P. Brooks et al.   NATURE REVIEWS NEUROSCIENCE

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

(2009) Liliana Menalled et al.   NEUROBIOLOGY OF DISEASE

Progressive imbalance in the interaction between spatial and procedural memory systems in the R6/2 mouse model of Huntington’s disease

(2009) Alessandro Ciamei et al.   NEUROBIOLOGY OF LEARNING AND MEMORY

Of mice, rats and men: Revisiting the quinolinic acid hypothesis of Huntington's disease

(2009) Robert Schwarcz et al.   PROGRESS IN NEUROBIOLOGY

Rapid Eye Movement Sleep Disturbances in Huntington Disease

(2008) Isabelle Arnulf et al.   ARCHIVES OF NEUROLOGY

The detection and measurement of locomotor deficits in a transgenic mouse model of Huntington's disease are task- and protocol-dependent: Influence of non-motor factors on locomotor function

(2008) Patrick N. Pallier et al.   BRAIN RESEARCH BULLETIN

Applications of Lentiviral Vectors for Biology and Gene Therapy of Neurological Disorders

(2008) Cecilia Lundberg et al.   CURRENT GENE THERAPY

Altered serotonin receptor expression is associated with depression-related behavior in the R6/1 transgenic mouse model of Huntington's disease

(2008) Terence Y.C. Pang et al.   HUMAN MOLECULAR GENETICS

Sex differences in a transgenic rat model of Huntington's disease: decreased 17β-estradiol levels correlate with reduced numbers of DARPP32+ neurons in males

(2008) Felix J. Bode et al.   HUMAN MOLECULAR GENETICS

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

(2008) Maria Björkqvist et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Full-Length Human Mutant Huntingtin with a Stable Polyglutamine Repeat Can Elicit Progressive and Selective Neuropathogenesis in BACHD Mice

(2008) M. Gray et al.   JOURNAL OF NEUROSCIENCE

Towards a transgenic model of Huntington’s disease in a non-human primate

(2008) Shang-Hsun Yang et al.   NATURE

Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice

(2008) A Jennifer Morton et al.   NEUROBIOLOGY OF DISEASE

CAG repeat lengths ≥335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse

(2008) I. Dragatsis et al.   NEUROBIOLOGY OF DISEASE

Weight loss in Huntington disease increases with higher CAG repeat number

(2008) N. A. Aziz et al.   NEUROLOGY

Suppression of Neurodegeneration and Increased Neurotransmission Caused by Expanded Full-Length Huntingtin Accumulating in the Cytoplasm

(2008) Eliana Romero et al.   NEURON

Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice

(2008) M.A. Hickey et al.   NEUROSCIENCE

DNA instability in postmitotic neurons

(2008) R. Gonitel et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Brain gene expression correlates with changes in behavior in the R6/1 mouse model of Huntington’s disease

(2007) A. Hodges et al.   GENES BRAIN AND BEHAVIOR

Detection of Huntington's disease decades before diagnosis: the Predict-HD study

(2007) J S Paulsen et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Gene–environment interactions modulating cognitive function and molecular correlates of synaptic plasticity in Huntington’s disease transgenic mice

(2007) Jess Nithianantharajah et al.   NEUROBIOLOGY OF DISEASE

Remodeling the model organism: Matrix metalloproteinase functions in invertebrates

(2007) A PAGEMCCAW   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY