Published in J Neurosci on December 01, 1999
Human umbilical cord plasma proteins revitalize hippocampal function in aged mice. Nature (2017) 2.71
Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models. Neurobiol Dis (2009) 2.29
The power of automated high-resolution behavior analysis revealed by its application to mouse models of Huntington's and prion diseases. Proc Natl Acad Sci U S A (2007) 1.99
The corticostriatal pathway in Huntington's disease. Prog Neurobiol (2006) 1.99
A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo. Proc Natl Acad Sci U S A (2005) 1.88
Transcriptional signatures in Huntington's disease. Prog Neurobiol (2007) 1.86
Huntington's disease: can mice lead the way to treatment? Neuron (2011) 1.71
Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function. Neuroscience (2011) 1.62
Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175. PLoS One (2012) 1.53
Choosing an animal model for the study of Huntington's disease. Nat Rev Neurosci (2013) 1.47
Up-regulating BDNF with an ampakine rescues synaptic plasticity and memory in Huntington's disease knockin mice. Proc Natl Acad Sci U S A (2009) 1.44
The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies. NeuroRx (2005) 1.33
Dysregulated information processing by medium spiny neurons in striatum of freely behaving mouse models of Huntington's disease. J Neurophysiol (2008) 1.28
Alterations in cortical excitation and inhibition in genetic mouse models of Huntington's disease. J Neurosci (2009) 1.28
Differential vulnerability of neurons in Huntington's disease: the role of cell type-specific features. J Neurochem (2010) 1.25
The antidepressant sertraline improves the phenotype, promotes neurogenesis and increases BDNF levels in the R6/2 Huntington's disease mouse model. Exp Neurol (2007) 1.22
CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse. Neurobiol Dis (2008) 1.19
Altered information processing in the prefrontal cortex of Huntington's disease mouse models. J Neurosci (2008) 1.18
Genetic knock-down of HDAC7 does not ameliorate disease pathogenesis in the R6/2 mouse model of Huntington's disease. PLoS One (2009) 1.15
Alterations in striatal synaptic transmission are consistent across genetic mouse models of Huntington's disease. ASN Neuro (2010) 1.09
Genetic mouse models of Huntington's disease: focus on electrophysiological mechanisms. ASN Neuro (2010) 1.03
Phosphodiesterases as therapeutic targets for Alzheimer's disease. ACS Chem Neurosci (2012) 1.03
Genetic knock-down of HDAC3 does not modify disease-related phenotypes in a mouse model of Huntington's disease. PLoS One (2012) 1.03
Circadian Abnormalities in Motor Activity in a BAC Transgenic Mouse Model of Huntington's Disease. PLoS Curr (2011) 1.00
Oral administration of the pimelic diphenylamide HDAC inhibitor HDACi 4b is unsuitable for chronic inhibition of HDAC activity in the CNS in vivo. PLoS One (2012) 0.98
Responses to environmental enrichment differ with sex and genotype in a transgenic mouse model of Huntington's disease. PLoS One (2010) 0.97
Molecular characterization of skeletal muscle atrophy in the R6/2 mouse model of Huntington's disease. Am J Physiol Endocrinol Metab (2011) 0.97
Skeletal muscle pathology in Huntington's disease. Front Physiol (2014) 0.94
Cell therapy in Huntington's disease. NeuroRx (2004) 0.94
Neurodevelopmental impairment following neonatal hyperoxia in the mouse. Neurobiol Dis (2012) 0.92
A ketogenic diet delays weight loss and does not impair working memory or motor function in the R6/2 1J mouse model of Huntington's disease. Physiol Behav (2011) 0.92
High-Throughput Automated Phenotyping of Two Genetic Mouse Models of Huntington's Disease. PLoS Curr (2013) 0.91
Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction. Proc Natl Acad Sci U S A (2013) 0.91
Direct evidence of progressive cardiac dysfunction in a transgenic mouse model of Huntington's disease. J Huntingtons Dis (2012) 0.90
Abnormal motor cortex plasticity in premanifest and very early manifest Huntington disease. J Neurol Neurosurg Psychiatry (2009) 0.90
A Longitudinal Operant Assessment of Cognitive and Behavioural Changes in the HdhQ111 Mouse Model of Huntington's Disease. PLoS One (2016) 0.89
Atypical diabetes associated with inclusion formation in the R6/2 mouse model of Huntington's disease is not improved by treatment with hypoglycaemic agents. Exp Brain Res (2005) 0.88
Impaired learning and memory in Pitx3 deficient aphakia mice: a genetic model for striatum-dependent cognitive symptoms in Parkinson's disease. Neurobiol Dis (2008) 0.87
Asialoerythropoietin is not effective in the R6/2 line of Huntington's disease mice. BMC Neurosci (2004) 0.87
CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease. Hum Mol Genet (2010) 0.86
Cognitive flexibility and long-term depression (LTD) are impaired following β-catenin stabilization in vivo. Proc Natl Acad Sci U S A (2014) 0.84
Impaired alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor trafficking and function by mutant huntingtin. J Biol Chem (2011) 0.84
Temporal separation of aggregation and ubiquitination during early inclusion formation in transgenic mice carrying the Huntington's disease mutation. PLoS One (2012) 0.84
Mouse models of polyglutamine diseases: review and data table. Part I. Mol Neurobiol (2012) 0.84
Dopamine Receptors and Neurodegeneration. Aging Dis (2015) 0.83
Huntington's disease mouse models online: high-resolution MRI images with stereotaxic templates for computational neuroanatomy. PLoS One (2012) 0.83
Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability. PLoS One (2011) 0.83
Reversal learning and associative memory impairments in a BACHD rat model for Huntington disease. PLoS One (2013) 0.81
Deficits in a Simple Visual Go/No-go Discrimination Task in Two Mouse Models of Huntington's Disease. PLoS Curr (2013) 0.81
Reduced expression of conditioned fear in the R6/2 mouse model of Huntington's disease is related to abnormal activity in prelimbic cortex. Neurobiol Dis (2011) 0.81
Glial A30P alpha-synuclein pathology segregates neurogenesis from anxiety-related behavior in conditional transgenic mice. Neurobiol Dis (2013) 0.81
HD mouse models reveal clear deficits in learning to perform a simple instrumental response. PLoS Curr (2011) 0.81
Roles of tau protein in health and disease. Acta Neuropathol (2017) 0.80
Somatostatin receptor 1 and 5 double knockout mice mimic neurochemical changes of Huntington's disease transgenic mice. PLoS One (2011) 0.79
Disruption of axonal transport perturbs bone morphogenetic protein (BMP)--signaling and contributes to synaptic abnormalities in two neurodegenerative diseases. PLoS One (2014) 0.79
The utilisation of operant delayed matching and non-matching to position for probing cognitive flexibility and working memory in mouse models of Huntington's disease. J Neurosci Methods (2015) 0.79
Studies on the Q175 Knock-in Model of Huntington's Disease Using Functional Imaging in Awake Mice: Evidence of Olfactory Dysfunction. Front Neurol (2014) 0.78
Right ventricular dysfunction in the R6/2 transgenic mouse model of Huntington's disease is unmasked by dobutamine. J Huntingtons Dis (2014) 0.78
Transplantation of induced pluripotent stem cells improves functional recovery in Huntington's disease rat model. PLoS One (2014) 0.77
Early retinal function deficit without prominent morphological changes in the R6/2 mouse model of Huntington's disease. PLoS One (2014) 0.77
Gabapentin-lactam, but not gabapentin, reduces protein aggregates and improves motor performance in a transgenic mouse model of Huntington's disease. Naunyn Schmiedebergs Arch Pharmacol (2004) 0.75
Genetic deletion of the Histone Deacetylase 6 exacerbates selected behavioral deficits in the R6/1 mouse model for Huntington's disease. Brain Behav (2015) 0.75
Similar striatal gene expression profiles in the striatum of the YAC128 and HdhQ150 mouse models of Huntington's disease are not reflected in mutant Huntingtin inclusion prevalence. BMC Genomics (2015) 0.75
Adaptation to experimental jet-lag in R6/2 mice despite circadian dysrhythmia. PLoS One (2013) 0.75
Impairment of blood-brain barrier is an early event in R6/2 mouse model of Huntington Disease. Sci Rep (2017) 0.75
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell (1996) 12.59
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science (1997) 11.53
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell (1997) 9.57
A candidate for the cystic fibrosis locus isolated by selection for methylation-free islands. Nature (1987) 7.88
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell (1997) 7.59
Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology. Proc Natl Acad Sci U S A (1999) 4.58
Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation. J Neurosci (1999) 4.56
The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A (2000) 4.01
A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis. Nat Genet (1998) 3.24
Knowledge of HIV antibody status and decisions to continue or terminate pregnancy among intravenous drug users. JAMA (1989) 2.41
Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. Nat Genet (1997) 2.37
Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene. Proc Natl Acad Sci U S A (1998) 2.14
Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease. Proc Natl Acad Sci U S A (2000) 2.00
Long-term survival of human central nervous system progenitor cells transplanted into a rat model of Parkinson's disease. Exp Neurol (1997) 1.98
Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol Dis (1998) 1.96
The "staircase test": a measure of independent forelimb reaching and grasping abilities in rats. J Neurosci Methods (1991) 1.93
Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. Hum Mol Genet (1999) 1.86
Unilateral transplantation of human primary fetal tissue in four patients with Huntington's disease: NEST-UK safety report ISRCTN no 36485475. J Neurol Neurosurg Psychiatry (2002) 1.76
Differential expression of immediate early genes in the hippocampus and spinal cord. Neuron (1990) 1.76
Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation. J Neurosci (2000) 1.75
Voxel-based morphometry in the R6/2 transgenic mouse reveals differences between genotypes not seen with manual 2D morphometry. Neurobiol Dis (2008) 1.73
Impaired glutamate uptake in the R6 Huntington's disease transgenic mice. Neurobiol Dis (2001) 1.71
Functional integration of striatal allografts in a primate model of Huntington's disease. Nat Med (1998) 1.71
Formation of polyglutamine inclusions in non-CNS tissue. Hum Mol Genet (1999) 1.70
Structure and expression of the Huntington's disease gene: evidence against simple inactivation due to an expanded CAG repeat. Somat Cell Mol Genet (1994) 1.66
SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates. Mol Cell (1998) 1.64
Spatial learning and motor deficits in aged rats. Neurobiol Aging (1984) 1.60
Sequence comparison of human and yeast telomeres identifies structurally distinct subtelomeric domains. Hum Mol Genet (1997) 1.57
Microdissection of and microcloning from the short arm of human chromosome 2. Mol Cell Biol (1986) 1.55
A candidate reference method for determination of total protein in serum. I. Development and validation. Clin Chem (1981) 1.52
Outbreak of Legionnaires' disease among cruise ship passengers exposed to a contaminated whirlpool spa. Lancet (1996) 1.50
Heat exhaustion in a deep underground metalliferous mine. Occup Environ Med (2000) 1.49
Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients. Hum Mol Genet (2001) 1.42
The contributions of motor cortex, nigrostriatal dopamine and caudate-putamen to skilled forelimb use in the rat. Brain (1986) 1.40
Motor coordination and balance in rodents. Curr Protoc Neurosci (2001) 1.39
Cell therapy in Parkinson's disease - stop or go? Nat Rev Neurosci (2001) 1.34
Impairments in the acquisition, retention and selection of spatial navigation strategies after medial caudate-putamen lesions in rats. Behav Brain Res (1987) 1.29
Structure of an RNA internal loop consisting of tandem C-A+ base pairs. Biochemistry (1998) 1.27
Spontaneous regression of human melanoma/nonmelanoma skin cancer: association with infiltrating CD4+ T cells. World J Surg (1995) 1.26
The huntingtin interacting protein HIP1 is a clathrin and alpha-adaptin-binding protein involved in receptor-mediated endocytosis. Hum Mol Genet (2001) 1.25
Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles. Neurobiol Dis (2001) 1.23
Stable transfection of calbindin-D28k into the GH3 cell line alters calcium currents and intracellular calcium homeostasis. Neuron (1992) 1.22
Dissociable roles of the ventral, medial and lateral striatum on the acquisition and performance of a complex visual stimulus-response habit. Behav Brain Res (1991) 1.22
Reinnervation of the denervated striatum by substantia nigra transplants: functional consequences as revealed by pharmacological and sensorimotor testing. Brain Res (1980) 1.21
Staging and preparation of human fetal striatal tissue for neural transplantation in Huntington's disease. Cell Transplant (2003) 1.20
Fluid losses and hydration status of industrial workers under thermal stress working extended shifts. Occup Environ Med (2003) 1.18
Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease. Philos Trans R Soc Lond B Biol Sci (1999) 1.18
Survival and differentiation of rat and human epidermal growth factor-responsive precursor cells following grafting into the lesioned adult central nervous system. Exp Neurol (1996) 1.18
Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? Lancet (1998) 1.17
Multiple partners and partner choice as risk factors for sexually transmitted disease among female college students. Sex Transm Dis (1992) 1.16
A zinc-finger gene ZNF141 mapping at 4p16.3/D4S90 is a candidate gene for the Wolf-Hirschhorn (4p-) syndrome. Hum Mol Genet (1993) 1.15
The organization of the human complement factor I gene (IF): a member of the serine protease gene family. Genomics (1994) 1.13
Behavioural profiles of inbred mouse strains used as transgenic backgrounds. II: cognitive tests. Genes Brain Behav (2005) 1.12
Comparative effects of cholinergic drugs and lesions of nucleus basalis or fimbria-fornix on delayed matching in rats. Psychopharmacology (Berl) (1985) 1.11
Mapping of cosmid clones in Huntington's disease region of chromosome 4. Somat Cell Mol Genet (1991) 1.11
Attentional functions of the forebrain cholinergic systems: effects of intraventricular hemicholinium, physostigmine, basal forebrain lesions and intracortical grafts on a multiple-choice serial reaction time task. Exp Brain Res (1992) 1.10
Evaluation of 37 AIDS prevention projects: successful approaches and barriers to program effectiveness. Health Educ Q (1996) 1.09
Detection of polyglutamine aggregation in mouse models. Methods Enzymol (1999) 1.09
Effects of STN lesions on simple vs choice reaction time tasks in the rat: preserved motor readiness, but impaired response selection. Eur J Neurosci (2001) 1.08
Tissue transplants for PD. Neurology (1994) 1.07
Use of magnetic resonance imaging for anatomical phenotyping of the R6/2 mouse model of Huntington's disease. Neurobiol Dis (2008) 1.06
Microfluidic chip-based synthesis of alginate microspheres for encapsulation of immortalized human cells. Biomicrofluidics (2007) 1.06
Progressive formation of inclusions in the striatum and hippocampus of mice transgenic for the human Huntington's disease mutation. J Neurocytol (2000) 1.06
Septal transplants restore maze learning in rats with fornix-fimbria lesions. Brain Res (1982) 1.06
Altered mitogen-activated protein kinase signaling, tau hyperphosphorylation and mild spatial learning dysfunction in transgenic rats expressing the beta-amyloid peptide intracellularly in hippocampal and cortical neurons. Neuroscience (2004) 1.05
The direct screening of cosmid libraries with YAC clones. Nucleic Acids Res (1991) 1.04
Dopamine-dependent long term potentiation in the dorsal striatum is reduced in the R6/2 mouse model of Huntington's disease. Neuroscience (2007) 1.03
Unilateral lesions of the dorsal striatum in rats disrupt responding in egocentric space. J Neurosci (1997) 1.02
The time course of loss of dopaminergic neurons and the gliotic reaction surrounding grafts of embryonic mesencephalon to the striatum. Exp Neurol (1996) 1.01
Function recovery following neural transplantation of embryonic septal nuclei in adult rats with septohippocampal lesions. Nature (1982) 1.01
Survival, neuronal differentiation, and fiber outgrowth of propagated human neural precursor grafts in an animal model of Huntington's disease. Cell Transplant (2000) 1.00
Operant-based instrumental learning for analysis of genetically modified models of Huntington's disease. Brain Res Bull (2011) 0.99
Amyloid-like inclusions in Huntington's disease. Neuroscience (2000) 0.99
The staircase test of skilled reaching in mice. Brain Res Bull (2001) 0.99
Hand tapping: a simple, reproducible, objective marker of motor dysfunction in Huntington's disease. J Neurol (2008) 0.99
Behavioral and neurochemical evaluation of a transgenic mouse model of Lesch-Nyhan syndrome. J Neurol Sci (1988) 0.99
The risk of heat exhaustion at a deep underground metalliferous mine in relation to surface temperatures. Occup Med (Lond) (2000) 0.98
Time-lapse analysis of aggregate formation in an inducible PC12 cell model of Huntington's disease reveals time-dependent aggregate formation that transiently delays cell death. Brain Res Bull (2007) 0.98
Striatal transplantation in a transgenic mouse model of Huntington's disease. Exp Neurol (1998) 0.98
HAP1-huntingtin interactions do not contribute to the molecular pathology in Huntington's disease transgenic mice. FEBS Lett (1998) 0.98
Effects of regional striatal lesions on motor, motivational, and executive aspects of progressive-ratio performance in rats. Behav Neurosci (1999) 0.98
Cross-species neural grafting in a rat model of Parkinson's disease. Nature (1982) 0.98
Efferent synaptic connections of grafted dopaminergic neurons reinnervating the host neostriatum: a tyrosine hydroxylase immunocytochemical study. J Neurosci (1985) 0.98
Environmental stimulation increases survival in mice transgenic for exon 1 of the Huntington's disease gene. Mov Disord (2000) 0.97
The telomeric 60 kb of chromosome arm 4p is homologous to telomeric regions on 13p, 15p, 21p, and 22p. Genomics (1992) 0.97
Transgenic mice for the amyloid precursor protein 695 isoform have impaired spatial memory. Neuroreport (1991) 0.97
Age-related impairments in spatial memory are independent of those in sensorimotor skills. Neurobiol Aging (1989) 0.97
Dopamine depletion, stimulation or blockade in the rat disrupts spatial navigation and locomotion dependent upon beacon or distal cues. Behav Brain Res (1986) 0.96
The risk of heat exhaustion at a deep underground metalliferous mine in relation to body-mass index and predicted VO2max. Occup Med (Lond) (2000) 0.95
Dopamine and cholecystokinin immunoreactive neurons in mesencephalic grafts reinnervating the neostriatum: evidence for selective growth regulation. Neuroscience (1984) 0.95
IgA multiple myeloma presenting as an acquired bullous disorder. Australas J Dermatol (1999) 0.95
The expression of Huntingtin-associated protein (HAP1) mRNA in developing, adult and ageing rat CNS: implications for Huntington's disease neuropathology. Eur J Neurosci (1998) 0.95
Selective immunolesioning of the basal forebrain cholinergic system disrupts short-term memory in rats. Eur J Neurosci (1996) 0.94