Published in J Proteome Res on June 07, 2007
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Does neuroinflammation fan the flame in neurodegenerative diseases? Mol Neurodegener (2009) 2.23
Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. J Clin Invest (2015) 1.74
Huntington's disease: the case for genetic modifiers. Genome Med (2009) 1.25
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
Abnormal peripheral chemokine profile in Huntington's disease. PLoS Curr (2011) 1.12
Mutant huntingtin impairs immune cell migration in Huntington disease. J Clin Invest (2012) 1.11
Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression. Hum Mol Genet (2010) 1.06
Huntington's disease: progress toward effective disease-modifying treatments and a cure. Hum Mol Genet (2010) 1.05
Bone marrow transplantation confers modest benefits in mouse models of Huntington's disease. J Neurosci (2012) 1.03
The role of the immune system in Huntington's disease. Clin Dev Immunol (2013) 1.03
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
Forkhead box protein p1 is a transcriptional repressor of immune signaling in the CNS: implications for transcriptional dysregulation in Huntington disease. Hum Mol Genet (2012) 0.96
Brain-specific proteins decline in the cerebrospinal fluid of humans with Huntington disease. Mol Cell Proteomics (2008) 0.96
Imaging of microglia in patients with neurodegenerative disorders. Front Pharmacol (2012) 0.96
Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis. FEBS J (2013) 0.90
Huntington's disease biomarker progression profile identified by transcriptome sequencing in peripheral blood. Eur J Hum Genet (2015) 0.88
Application of "omics" to prion biomarker discovery. J Biomed Biotechnol (2010) 0.86
Molecular diagnostics of neurodegenerative disorders. Front Mol Biosci (2015) 0.86
Peripheral inflammation in neurodegeneration. J Mol Med (Berl) (2013) 0.85
The choreography of neuroinflammation in Huntington's disease. Trends Immunol (2015) 0.85
The role of microglia in human disease: therapeutic tool or target? Acta Neuropathol (2014) 0.85
The role of the immune system in triplet repeat expansion diseases. Mediators Inflamm (2015) 0.85
Mouse models of polyglutamine diseases: review and data table. Part I. Mol Neurobiol (2012) 0.84
Whole gene expression profile in blood reveals multiple pathways deregulation in R6/2 mouse model. Biomark Res (2013) 0.83
Application of mass spectrometry-based proteomics for biomarker discovery in neurological disorders. Ann Indian Acad Neurol (2009) 0.82
JAK/STAT Signalling in Huntington's Disease Immune Cells. PLoS Curr (2013) 0.82
Increased prothrombin, apolipoprotein A-IV, and haptoglobin in the cerebrospinal fluid of patients with Huntington's disease. PLoS One (2011) 0.81
Activated microglia proliferate at neurites of mutant huntingtin-expressing neurons. Neurobiol Aging (2011) 0.81
Dysfunctional kynurenine pathway metabolism in the R6/2 mouse model of Huntington's disease. J Neurochem (2010) 0.81
Complement and HIV-I infection/HIV-associated neurocognitive disorders. J Neurovirol (2014) 0.80
Changes in the striatal proteome of YAC128Q mice exhibit gene-environment interactions between mutant huntingtin and manganese. J Proteome Res (2012) 0.80
Prospects for neuroprotective therapies in prodromal Huntington's disease. Mov Disord (2014) 0.80
Downregulation of genes involved in metabolism and oxidative stress in the peripheral leukocytes of Huntington's disease patients. PLoS One (2012) 0.78
Genetic Deficiency of Complement Component 3 Does Not Alter Disease Progression in a Mouse Model of Huntington's Disease. J Huntingtons Dis (2012) 0.78
Selected CSF biomarkers indicate no evidence of early neuroinflammation in Huntington disease. Neurol Neuroimmunol Neuroinflamm (2016) 0.78
The unfolded protein response and its potential role in Huntington ´s disease elucidated by a systems biology approach. F1000Res (2015) 0.77
Laquinimod dampens hyperactive cytokine production in Huntington's disease patient myeloid cells. J Neurochem (2016) 0.77
Cardiac Dysfunction in the BACHD Mouse Model of Huntington's Disease. PLoS One (2016) 0.77
Serum levels of a subset of cytokines show high interindividual variability and are not altered in rats transgenic for Huntington´s disease. PLoS Curr (2010) 0.77
Huntington's Disease: An Immune Perspective. Neurol Res Int (2011) 0.76
AAV-dominant negative tumor necrosis factor (DN-TNF) gene transfer to the striatum does not rescue medium spiny neurons in the YAC128 mouse model of Huntington's disease. PLoS One (2014) 0.76
The Effects of Pharmacological Inhibition of Histone Deacetylase 3 (HDAC3) in Huntington's Disease Mice. PLoS One (2016) 0.75
Neural and mesenchymal stem cells in animal models of Huntington's disease: past experiences and future challenges. Stem Cell Res Ther (2015) 0.75
Genomic and Proteomic Biomarker Discovery in Neurological Disease. Biomark Insights (2008) 0.75
Molecular Imaging Markers to Track Huntington's Disease Pathology. Front Neurol (2017) 0.75
Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies. Mediators Inflamm (2016) 0.75
Microglial Activation in the Pathogenesis of Huntington's Disease. Front Aging Neurosci (2017) 0.75
Synergistic toxicity of polyglutamine-expanded TBP in glia and neuronal cells: therapeutic implications for SCA17. J Neurosci (2017) 0.75
β-Defensin Genomic Copy Number Does Not Influence the Age of Onset in Huntington's Disease. J Huntingtons Dis (2013) 0.75
Beneficial effects of glatiramer acetate in Huntington's disease mouse models: evidence for BDNF-elevating and immunomodulatory mechanisms. Brain Res (2017) 0.75
Huntington's disease: from molecular pathogenesis to clinical treatment. Lancet Neurol (2011) 4.98
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat Genet (2003) 4.76
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell (2006) 4.53
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
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci (2002) 4.15
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet (2003) 4.07
Global changes to the ubiquitin system in Huntington's disease. Nature (2007) 3.91
Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data. Lancet Neurol (2009) 3.82
Association of plasma clusterin concentration with severity, pathology, and progression in Alzheimer disease. Arch Gen Psychiatry (2010) 3.64
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. Lancet Neurol (2013) 3.56
Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis. Lancet Neurol (2010) 3.47
PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death. Mol Cell (2009) 3.17
Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron (2002) 3.15
Revised national guidelines for analysis of cerebrospinal fluid for bilirubin in suspected subarachnoid haemorrhage. Ann Clin Biochem (2008) 3.07
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay. Lancet (2011) 2.60
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
Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat Cell Biol (2002) 2.50
Antiviral protein APOBEC3G localizes to ribonucleoprotein complexes found in P bodies and stress granules. J Virol (2006) 2.50
Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series. EMBO Rep (2004) 2.35
The clinical significance of an intrathecal monoclonal immunoglobulin band: a follow-up study. Neurology (2003) 2.35
PINK1 is necessary for long term survival and mitochondrial function in human dopaminergic neurons. PLoS One (2008) 2.33
Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease. Proc Natl Acad Sci U S A (2013) 2.21
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
Large C9orf72 hexanucleotide repeat expansions are seen in multiple neurodegenerative syndromes and are more frequent than expected in the UK population. Am J Hum Genet (2013) 2.09
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
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. J Neurosci (2002) 2.05
Disease-associated prion protein oligomers inhibit the 26S proteasome. Mol Cell (2007) 2.01
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
Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study. Lancet Neurol (2009) 1.94
Microglial activation in presymptomatic Huntington's disease gene carriers. Brain (2007) 1.93
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
Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc Natl Acad Sci U S A (2005) 1.87
Beyond the brain: widespread pathology in Huntington's disease. Lancet Neurol (2009) 1.83
Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease. J Neurosci (2005) 1.82
HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum Mol Genet (2002) 1.79
Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin. Biochem J (2008) 1.77
Environmental enrichment slows disease progression in R6/2 Huntington's disease mice. Ann Neurol (2002) 1.74
Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. J Clin Invest (2015) 1.74
Interleukin-6 is elevated in the cerebrospinal fluid of suicide attempters and related to symptom severity. Biol Psychiatry (2009) 1.72
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
Tetrabenazine. Nat Rev Drug Discov (2009) 1.60
Phosphorylation of huntingtin at Ser421 in YAC128 neurons is associated with protection of YAC128 neurons from NMDA-mediated excitotoxicity and is modulated by PP1 and PP2A. J Neurosci (2010) 1.58
Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease. J Neurochem (2007) 1.54
Gene expression in Huntington's disease skeletal muscle: a potential biomarker. Hum Mol Genet (2005) 1.51
White matter connections reflect changes in voluntary-guided saccades in pre-symptomatic Huntington's disease. Brain (2007) 1.47
Observing Huntington's Disease: the European Huntington's Disease Network's REGISTRY. PLoS Curr (2010) 1.45
Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease. J Neurosci (2009) 1.43
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
Suppression of protein aggregation by chaperone modification of high molecular weight complexes. Brain (2012) 1.42
Clinical signs and CRP values associated with blood culture results in neonates evaluated for suspected sepsis. Acta Paediatr (2010) 1.42
Disease-related prion protein forms aggresomes in neuronal cells leading to caspase activation and apoptosis. J Biol Chem (2005) 1.42
Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease. J Neurosci (2009) 1.40
The Prevalence of Huntington's Disease. Neuroepidemiology (2016) 1.40
Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease. Hum Mol Genet (2005) 1.40
Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease. Hum Mol Genet (2005) 1.39
Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease. Hum Mol Genet (2005) 1.39
Early changes in white matter pathways of the sensorimotor cortex in premanifest Huntington's disease. Hum Brain Mapp (2011) 1.39
CAG-encoded polyglutamine length polymorphism in the human genome. BMC Genomics (2007) 1.36
Huntington's disease. BMJ (2010) 1.36
Analysis of potential transcriptomic biomarkers for Huntington's disease in peripheral blood. Proc Natl Acad Sci U S A (2007) 1.35
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
Orexin loss in Huntington's disease. Hum Mol Genet (2004) 1.33
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
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
Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records. J Neurol Neurosurg Psychiatry (2013) 1.30
AddNeuroMed--the European collaboration for the discovery of novel biomarkers for Alzheimer's disease. Ann N Y Acad Sci (2009) 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
Isolation and characterisation of potential respiratory syncytial virus receptor(s) on epithelial cells. Microbes Infect (2003) 1.28
Development of biomarkers for Huntington's disease. Lancet Neurol (2011) 1.26
Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain. Neurobiol Dis (2006) 1.25
Hsa-miR-34b is a plasma-stable microRNA that is elevated in pre-manifest Huntington's disease. Hum Mol Genet (2011) 1.25