Published in PLoS One on April 04, 2013
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A common gene expression signature in Huntington's disease patient brain regions. BMC Med Genomics (2014) 0.88
Treatment with a herbal formula B401 enhances neuroprotection and angiogenesis in the R6/2 mouse model of Huntington's disease. Drug Des Devel Ther (2015) 0.83
Analysis of YFP(J16)-R6/2 reporter mice and postmortem brains reveals early pathology and increased vulnerability of callosal axons in Huntington's disease. Hum Mol Genet (2015) 0.81
Neurorestoration induced by the HDAC inhibitor sodium valproate in the lactacystin model of Parkinson's is associated with histone acetylation and up-regulation of neurotrophic factors. Br J Pharmacol (2015) 0.80
Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's disease. PLoS One (2013) 0.79
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Progressive Cl- channel defects reveal disrupted skeletal muscle maturation in R6/2 Huntington's mice. J Gen Physiol (2016) 0.78
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Electrophysiological and morphological changes in striatal spiny neurons in R6/2 Huntington's disease transgenic mice. J Neurophysiol (2001) 1.91
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Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease. Hum Mol Genet (2010) 1.71
Weight loss in Huntington disease increases with higher CAG repeat number. Neurology (2008) 1.66
Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease. J Clin Invest (2011) 1.66
Myelin breakdown and iron changes in Huntington's disease: pathogenesis and treatment implications. Neurochem Res (2007) 1.63
Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice. J Neurosci (2002) 1.48
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Early behavioral deficits in R6/2 mice suitable for use in preclinical drug testing. Neurobiol Dis (2005) 1.33
Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease. PLoS One (2009) 1.31
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Longitudinal characterization of brain atrophy of a Huntington's disease mouse model by automated morphological analyses of magnetic resonance images. Neuroimage (2009) 1.15
Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice. J Comp Neurol (2002) 1.14
Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease. J Neurol Neurosurg Psychiatry (2012) 1.11
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. Brain Res Bull (2008) 1.09
Sex differences in behavior and striatal ascorbate release in the 140 CAG knock-in mouse model of Huntington's disease. Behav Brain Res (2007) 1.09
Striatal volume contributes to the prediction of onset of Huntington disease in incident cases. Biol Psychiatry (2011) 1.09
Sex differences in a transgenic rat model of Huntington's disease: decreased 17beta-estradiol levels correlate with reduced numbers of DARPP32+ neurons in males. Hum Mol Genet (2008) 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
Increased basal ganglia iron levels in Huntington disease. Arch Neurol (1999) 1.01
Cognitive and non-cognitive behaviors in an APPswe/PS1 bigenic model of Alzheimer's disease. Genes Brain Behav (2008) 1.01
Alterations in brain transition metals in Huntington disease: an evolving and intricate story. Arch Neurol (2012) 0.99
Assessment of normal myelination with magnetic resonance imaging. Semin Neurol (2012) 0.99
Responses to environmental enrichment differ with sex and genotype in a transgenic mouse model of Huntington's disease. PLoS One (2010) 0.97
MRI T2 Hypointensities in basal ganglia of premanifest Huntington's disease. PLoS Curr (2010) 0.93
Elevated brain iron is independent from atrophy in Huntington's Disease. Neuroimage (2012) 0.93
Microglia density decreases with age in a mouse model of Huntington's disease. Glia (2003) 0.92
The structural correlates of functional deficits in early huntington's disease. Hum Brain Mapp (2012) 0.92
Cortical metabolites as biomarkers in the R6/2 model of Huntington's disease. J Cereb Blood Flow Metab (2011) 0.91
An open system for automatic home-cage behavioral analysis and its application to male and female mouse models of Huntington's disease. Behav Brain Res (2012) 0.86
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Loss of cortical and thalamic neuronal tenascin-C expression in a transgenic mouse expressing exon 1 of the human Huntington disease gene. J Comp Neurol (2001) 0.86
Relationship between BDNF expression in major striatal afferents, striatum morphology and motor behavior in the R6/2 mouse model of Huntington's disease. Genes Brain Behav (2012) 0.83
MR relaxometry in Huntington's disease: correlation between imaging, genetic and clinical parameters. J Neurol Sci (2007) 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
Spatiotemporal mapping of brain atrophy in mouse models of Huntington's disease using longitudinal in vivo magnetic resonance imaging. Neuroimage (2012) 0.79
Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease. Proc Natl Acad Sci U S A (2003) 4.44
Global changes to the ubiquitin system in Huntington's disease. Nature (2007) 3.91
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Minocycline and doxycycline are not beneficial in a model of Huntington's disease. Ann Neurol (2003) 2.57
Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum Mol Genet (2007) 2.54
Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series. EMBO Rep (2004) 2.35
Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease. Proc Natl Acad Sci U S A (2013) 2.21
MRI findings and sleep apnea in children with Chiari I malformation. Pediatr Neurol (2013) 2.13
Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease. J Biol Chem (2010) 2.10
SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis. Proc Natl Acad Sci U S A (2010) 2.09
Histone deacetylase inhibitors as therapeutics for polyglutamine disorders. Nat Rev Neurosci (2006) 2.07
The Hdh(Q150/Q150) knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes. Brain Res Bull (2006) 2.00
Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach. Hum Mol Genet (2004) 2.00
HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration. PLoS Biol (2013) 1.96
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
Environmental enrichment slows disease progression in R6/2 Huntington's disease mice. Ann Neurol (2002) 1.74
Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease. Hum Mol Genet (2010) 1.71
Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease. J Clin Invest (2011) 1.66
Polyglutamine expansion of huntingtin impairs its nuclear export. Nat Genet (2005) 1.62
DNA instability in postmitotic neurons. Proc Natl Acad Sci U S A (2008) 1.61
Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. J Proteome Res (2007) 1.59
Contrasting effects of haloperidol and lithium on rodent brain structure: a magnetic resonance imaging study with postmortem confirmation. Biol Psychiatry (2012) 1.56
Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease. J Neurochem (2007) 1.54
Effect of inflammatory cytokines on major histocompatibility complex expression and differentiation of human neural stem/progenitor cells. Stem Cells (2008) 1.51
Suppression of protein aggregation by chaperone modification of high molecular weight complexes. Brain (2012) 1.42
Exendin-4 improves glycemic control, ameliorates brain and pancreatic pathologies, and extends survival in a mouse model of Huntington's disease. Diabetes (2008) 1.42
Cavernous malformations of the brain after treatment for acute lymphocytic leukemia: presentation and long-term follow-up. J Neurosurg Pediatr (2012) 1.40
Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease. Hum Mol Genet (2005) 1.39
The support of neural stem cells transplanted into stroke-induced brain cavities by PLGA particles. Biomaterials (2009) 1.36
SAHA decreases HDAC 2 and 4 levels in vivo and improves molecular phenotypes in the R6/2 mouse model of Huntington's disease. PLoS One (2011) 1.34
Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease. PLoS One (2009) 1.31
Bone marrow-derived mesenchymal stem cells promote neuronal networks with functional synaptic transmission after transplantation into mice with neurodegeneration. Stem Cells (2007) 1.30
Proteasome impairment does not contribute to pathogenesis in R6/2 Huntington's disease mice: exclusion of proteasome activator REGgamma as a therapeutic target. Hum Mol Genet (2005) 1.30
Research capacity building: a US-South African partnership. Glob Health Promot (2011) 1.28
The in vitro effects of a bimodal contrast agent on cellular functions and relaxometry. NMR Biomed (2007) 1.23
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Optimisation of region-specific reference gene selection and relative gene expression analysis methods for pre-clinical trials of Huntington's disease. Mol Neurodegener (2008) 1.20
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Conservative treatment of spontaneous spinal epidural hematoma associated with oral anticoagulant therapy in a child. Childs Nerv Syst (2006) 1.18
A brain-permeable small molecule reduces neuronal cholesterol by inhibiting activity of sirtuin 2 deacetylase. ACS Chem Biol (2011) 1.17
Rotational stability of the AcrySof SA60TT toric intraocular lenses: a cohort study. BMC Ophthalmol (2008) 1.16
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
Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease. Hum Mol Genet (2006) 1.15
Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression. J Clin Invest (2012) 1.14
A prospective longitudinal model of substance use onset among South African adolescents. Subst Use Misuse (2009) 1.13
TR-FRET-based duplex immunoassay reveals an inverse correlation of soluble and aggregated mutant huntingtin in huntington's disease. Chem Biol (2012) 1.13
HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation. Brain (2014) 1.13
The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease. J Clin Invest (2011) 1.11
Increased metabolism in the R6/2 mouse model of Huntington's disease. Neurobiol Dis (2007) 1.11
Metabolic characterization of the R6/2 transgenic mouse model of Huntington's disease by high-resolution MAS 1H NMR spectroscopy. J Proteome Res (2006) 1.08
Hdac6 knock-out increases tubulin acetylation but does not modify disease progression in the R6/2 mouse model of Huntington's disease. PLoS One (2011) 1.07
Targeting the Nrf2-Keap1 antioxidant defence pathway for neurovascular protection in stroke. J Physiol (2011) 1.05
Attachment of stem cells to scaffold particles for intra-cerebral transplantation. Nat Protoc (2009) 1.04
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Sulforaphane preconditioning of the Nrf2/HO-1 defense pathway protects the cerebral vasculature against blood-brain barrier disruption and neurological deficits in stroke. Free Radic Biol Med (2013) 1.03
Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation. Hum Mol Genet (2007) 1.03
Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice. Neurobiol Dis (2006) 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
Hydrogels derived from central nervous system extracellular matrix. Biomaterials (2012) 1.02
Evaluation of the benzothiazole aggregation inhibitors riluzole and PGL-135 as therapeutics for Huntington's disease. Neurobiol Dis (2005) 1.02
A large number of protein expression changes occur early in life and precede phenotype onset in a mouse model for huntington disease. Mol Cell Proteomics (2008) 0.99
A human single-chain Fv intrabody preferentially targets amino-terminal Huntingtin's fragments in striatal models of Huntington's disease. Neurobiol Dis (2005) 0.99
Targeting H3K4 trimethylation in Huntington disease. Proc Natl Acad Sci U S A (2013) 0.98
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
Complex alteration of NMDA receptors in transgenic Huntington's disease mouse brain: analysis of mRNA and protein expression, plasma membrane association, interacting proteins, and phosphorylation. Neurobiol Dis (2003) 0.97
Abnormal phosphorylation of synapsin I predicts a neuronal transmission impairment in the R6/2 Huntington's disease transgenic mice. Mol Cell Neurosci (2002) 0.97
The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease. PLoS One (2009) 0.97
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Purmorphamine increases DARPP-32 differentiation in human striatal neural stem cells through the Hedgehog pathway. Stem Cells Dev (2011) 0.96
Neo-vascularization of the stroke cavity by implantation of human neural stem cells on VEGF-releasing PLGA microparticles. Biomaterials (2012) 0.96
Alterations in the mouse and human proteome caused by Huntington's disease. Mol Cell Proteomics (2002) 0.95
HDAC4 does not act as a protein deacetylase in the postnatal murine brain in vivo. PLoS One (2013) 0.94
Arfaptin 2 regulates the aggregation of mutant huntingtin protein. Nat Cell Biol (2002) 0.93
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Evolution of extra-nigral damage predicts behavioural deficits in a rat proteasome inhibitor model of Parkinson's disease. PLoS One (2011) 0.92
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Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease. Eur J Neurosci (2005) 0.90
Gastrointestinal dysfunction contributes to weight loss in Huntington's disease mice. Neurobiol Dis (2011) 0.89
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SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation. Cell Rep (2013) 0.88
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A web-based information system for management and analysis of patient data after refractive eye surgery. Comput Methods Programs Biomed (2007) 0.87
Meningioangiomatosis associated with meningioma: a case report. Acta Cytol (2009) 0.86
CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease. Hum Mol Genet (2010) 0.86
Biologic scaffold for CNS repair. Regen Med (2014) 0.84
Registration of challenging pre-clinical brain images. J Neurosci Methods (2013) 0.84
Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction. Neurobiol Dis (2005) 0.84
Downregulation of cannabinoid receptor 1 from neuropeptide Y interneurons in the basal ganglia of patients with Huntington's disease and mouse models. Eur J Neurosci (2012) 0.84
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Monitoring brain repair in stroke using advanced magnetic resonance imaging. Stroke (2012) 0.82
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A comparison of automated anatomical-behavioural mapping methods in a rodent model of stroke. J Neurosci Methods (2013) 0.82
Implantation of undifferentiated and pre-differentiated human neural stem cells in the R6/2 transgenic mouse model of Huntington's disease. BMC Neurosci (2012) 0.82
Characterization of the low pH/low nutrient-resistant LNCaP cell subline LNCaP-F10. Oncol Rep (2012) 0.81