An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes

Motor deficits associated with Huntington's disease occur in the absence of striatal degeneration in BACHD transgenic mice

(2016) Susanna Mantovani et al.   HUMAN MOLECULAR GENETICS

HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity

(2015) Virginia B. Mattis et al.   HUMAN MOLECULAR GENETICS

Altered excitatory and inhibitory inputs to striatal medium-sized spiny neurons and cortical pyramidal neurons in the Q175 mouse model of Huntington's disease

(2015) Tim Indersmitten et al.   JOURNAL OF NEUROPHYSIOLOGY

Aβ reduction in BACE1 heterozygous null 5XFAD mice is associated with transgenic APP level

(2015) Katherine R Sadleir et al.   Molecular Neurodegeneration

Therapeutic advances in Huntington's Disease

(2015) Kathleen M. Shannon et al.   MOVEMENT DISORDERS

Progressive axonal transport and synaptic protein changes correlate with behavioral and neuropathological abnormalities in the heterozygous Q175 KI mouse model of Huntington's disease

(2014) G. A. Smith et al.   HUMAN MOLECULAR GENETICS

Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records

(2013) Stephen JW Evans et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data

(2013) Sarah J Tabrizi et al.   LANCET NEUROLOGY

Multisource ascertainment of Huntington disease in Canada: Prevalence and population at risk

(2013) Emily R. Fisher et al.   MOVEMENT DISORDERS

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

(2013) Mahmoud A. Pouladi et al.   NATURE REVIEWS NEUROSCIENCE

Mismatch Repair Genes Mlh1 and Mlh3 Modify CAG Instability in Huntington's Disease Mice: Genome-Wide and Candidate Approaches

(2013) Ricardo Mouro Pinto et al.   PLoS Genetics

Induced Pluripotent Stem Cells from Patients with Huntington's Disease Show CAG-Repeat-Expansion-Associated Phenotypes

(2012) The HD iPSC Consortium   Cell Stem Cell

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

A touch screen-automated cognitive test battery reveals impaired attention, memory abnormalities, and increased response inhibition in the TgCRND8 mouse model of Alzheimer's disease

(2012) Carola Romberg et al.   NEUROBIOLOGY OF AGING

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

Characterization of Neurophysiological and Behavioral Changes, MRI Brain Volumetry and 1H MRS in zQ175 Knock-In Mouse Model of Huntington's Disease

(2012) Taneli Heikkinen et al.   PLoS One

Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease

(2011) Jeffrey B Carroll et al.   Molecular Neurodegeneration

Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice

(2011) Rocky G. Gogliotti et al.   NEUROBIOLOGY OF DISEASE

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

Huntington's disease: from molecular pathogenesis to clinical treatment

(2010) Christopher A Ross et al.   LANCET NEUROLOGY

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

(2010) Chiara Zuccato et al.   PHYSIOLOGICAL REVIEWS

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

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

Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin

(2008) Chuan-En Wang et al.   JOURNAL OF CELL BIOLOGY

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

Rodent genetic models of Huntington disease

(2008) Mary Y. Heng et al.   NEUROBIOLOGY OF DISEASE