Published in Brain on September 17, 2015
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Parkinson's disease and alpha synuclein: is Parkinson's disease a prion-like disorder? Mov Disord (2013) 1.34
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Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease. Brain (2012) 0.95
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White matter connectivity reflects clinical and cognitive status in Huntington's disease. Neurobiol Dis (2014) 0.90
The effects of SIFT on the reproducibility and biological accuracy of the structural connectome. Neuroimage (2014) 0.88
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Disconnection of network hubs and cognitive impairment after traumatic brain injury. Brain (2015) 0.86
Rich Club Organization and Cognitive Performance in Healthy Older Participants. J Cogn Neurosci (2015) 0.83
Basal ganglia-cortical structural connectivity in Huntington's disease. Hum Brain Mapp (2015) 0.81
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Microglial activation in presymptomatic Huntington's disease gene carriers. Brain (2007) 1.93
Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. J Clin Invest (2015) 1.74
Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. J Proteome Res (2007) 1.59
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Observing Huntington's Disease: the European Huntington's Disease Network's REGISTRY. PLoS Curr (2010) 1.45
Disease-related prion protein forms aggresomes in neuronal cells leading to caspase activation and apoptosis. J Biol Chem (2005) 1.42
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Early changes in white matter pathways of the sensorimotor cortex in premanifest Huntington's disease. Hum Brain Mapp (2011) 1.39
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Analysis of potential transcriptomic biomarkers for Huntington's disease in peripheral blood. Proc Natl Acad Sci U S A (2007) 1.35
Microglial activation in regions related to cognitive function predicts disease onset in Huntington's disease: a multimodal imaging study. Hum Brain Mapp (2011) 1.32
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Hsa-miR-34b is a plasma-stable microRNA that is elevated in pre-manifest Huntington's disease. Hum Mol Genet (2011) 1.25
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Plasma 24S-hydroxycholesterol and caudate MRI in pre-manifest and early Huntington's disease. Brain (2008) 1.16
Hypothalamic involvement in Huntington's disease: an in vivo PET study. Brain (2008) 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
Defective emotion recognition in early HD is neuropsychologically and anatomically generic. Neuropsychologia (2008) 1.15
Whole-brain atrophy as a measure of progression in premanifest and early Huntington's disease. Mov Disord (2009) 1.14
Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression. J Clin Invest (2012) 1.14
HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation. Brain (2014) 1.13
Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry. EMBO J (2011) 1.13
Abnormal peripheral chemokine profile in Huntington's disease. PLoS Curr (2011) 1.12
Imaging microglial activation in Huntington's disease. Brain Res Bull (2006) 1.12
Mutant huntingtin impairs immune cell migration in Huntington disease. J Clin Invest (2012) 1.11
Functional compensation of motor function in pre-symptomatic Huntington's disease. Brain (2009) 1.11
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Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease. Hum Mol Genet (2011) 1.10
The progression of regional atrophy in premanifest and early Huntington's disease: a longitudinal voxel-based morphometry study. J Neurol Neurosurg Psychiatry (2009) 1.09
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
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C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies. Neurology (2013) 1.06
Clinical impairment in premanifest and early Huntington's disease is associated with regionally specific atrophy. Hum Brain Mapp (2011) 1.05
An event-based model for disease progression and its application in familial Alzheimer's disease and Huntington's disease. Neuroimage (2012) 1.04
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Early atrophy of pallidum and accumbens nucleus in Huntington's disease. J Neurol (2010) 1.04
Brain-derived neurotrophic factor in patients with Huntington's disease. PLoS One (2011) 1.04
Bone marrow transplantation confers modest benefits in mouse models of Huntington's disease. J Neurosci (2012) 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
The application of NMR-based metabonomics in neurological disorders. NeuroRx (2006) 1.03
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Cannabinoid receptor 2 signaling in peripheral immune cells modulates disease onset and severity in mouse models of Huntington's disease. J Neurosci (2012) 1.00
Irritability in pre-clinical Huntington's disease. Neuropsychologia (2009) 0.99
Huntington's disease phenocopies are clinically and genetically heterogeneous. Mov Disord (2008) 0.99
Oculomotor deficits indicate the progression of Huntington's disease. Prog Brain Res (2008) 0.98
Expressed Alu repeats as a novel, reliable tool for normalization of real-time quantitative RT-PCR data. Genome Biol (2010) 0.97
Harnessing immune alterations in neurodegenerative diseases. Neuron (2009) 0.95
Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease. Brain (2012) 0.95
Alternative fates of newly formed PrPSc upon prion conversion on the plasma membrane. J Cell Sci (2013) 0.93
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The structural correlates of functional deficits in early huntington's disease. Hum Brain Mapp (2012) 0.92
Abnormal motor cortex excitability in preclinical and very early Huntington's disease. Biol Psychiatry (2009) 0.92
Stability effects on results of diffusion tensor imaging analysis by reduction of the number of gradient directions due to motion artifacts: an application to presymptomatic Huntington's disease. PLoS Curr (2011) 0.92
Visuomotor integration deficits precede clinical onset in Huntington's disease. Neuropsychologia (2010) 0.90
The ubiquitin-proteasome system in neurodegeneration. Antioxid Redox Signal (2014) 0.90
Automated quantification of caudate atrophy by local registration of serial MRI: evaluation and application in Huntington's disease. Neuroimage (2009) 0.90
Abnormal motor cortex plasticity in premanifest and very early manifest Huntington disease. J Neurol Neurosurg Psychiatry (2009) 0.90
The structural involvement of the cingulate cortex in premanifest and early Huntington's disease. Mov Disord (2011) 0.90
Relationship between CAG repeat length and brain volume in premanifest and early Huntington's disease. J Neurol (2009) 0.89
Spinocerebellar ataxia type 17: extension of phenotype with putaminal rim hyperintensity on magnetic resonance imaging. Mov Disord (2005) 0.89
Stability of white matter changes related to Huntington's disease in the presence of imaging noise: a DTI study. PLoS Curr (2011) 0.89
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Predict-HD and the future of therapeutic trials. Lancet Neurol (2006) 0.89
8OHdG is not a biomarker for Huntington disease state or progression. Neurology (2013) 0.88
A longitudinal study of magnetic resonance spectroscopy Huntington's disease biomarkers. Mov Disord (2015) 0.88
An ITPR1 gene deletion causes spinocerebellar ataxia 15/16: a genetic, clinical and radiological description. Mov Disord (2010) 0.88
Emotion recognition in Huntington's disease: a systematic review. Neurosci Biobehav Rev (2011) 0.88
Increased thirst and drinking in Huntington's disease and the R6/2 mouse. Brain Res Bull (2008) 0.88
Probabilistic classification learning with corrective feedback is selectively impaired in early Huntington's disease--evidence for the role of the striatum in learning with feedback. Neuropsychologia (2012) 0.88
Structural MRI in Huntington's disease and recommendations for its potential use in clinical trials. Neurosci Biobehav Rev (2013) 0.88
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NMDA receptor gene variations as modifiers in Huntington disease: a replication study. PLoS Curr (2011) 0.87
Peripheral inflammation in neurodegeneration. J Mol Med (Berl) (2013) 0.85
Abnormal explicit but normal implicit sequence learning in premanifest and early Huntington's disease. Mov Disord (2010) 0.85
Interregional compensatory mechanisms of motor functioning in progressing preclinical neurodegeneration. Neuroimage (2013) 0.85
Cortical dopamine dysfunction in symptomatic and premanifest Huntington's disease gene carriers. Neurobiol Dis (2009) 0.84
Prions and the proteasome. Biochim Biophys Acta (2008) 0.84
Plasma neurofilament heavy chain levels in Huntington's disease. Neurosci Lett (2007) 0.84
Mouse models as a tool for understanding neurodegenerative diseases. Curr Opin Neurol (2003) 0.84
An exploratory double-blind, randomized clinical trial with selisistat, a SirT1 inhibitor, in patients with Huntington's disease. Br J Clin Pharmacol (2015) 0.83
Misfolded PrP and a novel mechanism of proteasome inhibition. Prion (2012) 0.83
Saccadometry of conditional rules in presymptomatic Huntington's disease. Ann N Y Acad Sci (2009) 0.82
Development of an ELISA assay for the quantification of soluble huntingtin in human blood cells. BMC Biochem (2013) 0.82
Evaluation of multi-modal, multi-site neuroimaging measures in Huntington's disease: Baseline results from the PADDINGTON study. Neuroimage Clin (2012) 0.82
Basal ganglia-cortical structural connectivity in Huntington's disease. Hum Brain Mapp (2015) 0.81
Reliability and factor structure of the Short Problem Behaviors Assessment for Huntington's disease (PBA-s) in the TRACK-HD and REGISTRY studies. J Neuropsychiatry Clin Neurosci (2015) 0.81
Evaluating multicenter DTI data in Huntington's disease on site specific effects: An ex post facto approach. Neuroimage Clin (2013) 0.81
Emotional face recognition deficits and medication effects in pre-manifest through stage-II Huntington's disease. Psychiatry Res (2012) 0.81
Biomarkers for Huntington's disease: an update. Expert Opin Med Diagn (2012) 0.81