Published in Brain on July 01, 2007
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
Neurocognitive signs in prodromal Huntington disease. Neuropsychology (2011) 2.14
"Frontal" behaviors before the diagnosis of Huntington's disease and their relationship to markers of disease progression: evidence of early lack of awareness. J Neuropsychiatry Clin Neurosci (2010) 1.76
Cognitive impairment in Huntington disease: diagnosis and treatment. Curr Neurol Neurosci Rep (2011) 1.55
An ovine transgenic Huntington's disease model. Hum Mol Genet (2010) 1.24
Ten-year rate of longitudinal change in neurocognitive and motor function in prediagnosis Huntington disease. Mov Disord (2008) 1.16
Tetrabenazine: the first approved drug for the treatment of chorea in US patients with Huntington disease. Neuropsychiatr Dis Treat (2010) 1.06
Cognitive domains that predict time to diagnosis in prodromal Huntington disease. J Neurol Neurosurg Psychiatry (2012) 1.06
A review of quality of life after predictive testing for and earlier identification of neurodegenerative diseases. Prog Neurobiol (2013) 1.03
Irritability in pre-clinical Huntington's disease. Neuropsychologia (2009) 0.99
Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease. Brain (2012) 0.95
Emotional arousal and multiple memory systems in the mammalian brain. Front Behav Neurosci (2012) 0.95
Not on the face alone: perception of contextualized face expressions in Huntington's disease. Brain (2009) 0.92
Emotion recognition and experience in Huntington disease: a voxel-based morphometry study. J Psychiatry Neurosci (2011) 0.89
Excessive disgust caused by brain lesions or temporary inactivations: mapping hotspots of the nucleus accumbens and ventral pallidum. Eur J Neurosci (2014) 0.87
The neurology of disgust. Brain (2007) 0.86
Neuroanatomical correlates of cognitive functioning in prodromal Huntington disease. Brain Behav (2013) 0.85
Executive functions in premanifest Huntington's disease. Mov Disord (2013) 0.84
Risk-taking and pathological gambling behavior in Huntington's disease. Front Behav Neurosci (2014) 0.84
Facial emotion recognition impairments in individuals with HIV. J Int Neuropsychol Soc (2010) 0.83
Biomarkers to predict and track diseases. Lancet Neurol (2009) 0.83
Alexithymia and impaired facial affect recognition in multiple sclerosis. J Neurol (2011) 0.83
Emotion recognition and experience in Huntington's disease: is there a differential impairment? Psychiatry Res (2011) 0.82
Regional atrophy associated with cognitive and motor function in prodromal Huntington disease. J Huntingtons Dis (2013) 0.81
Depressive symptoms in prodromal Huntington's Disease correlate with Stroop-interference related functional connectivity in the ventromedial prefrontal cortex. Psychiatry Res (2012) 0.81
Neural correlates of impaired emotion processing in manifest Huntington's disease. Soc Cogn Affect Neurosci (2013) 0.80
Early Detection of Huntington Disease. Future Neurol (2010) 0.79
The role of the amygdala during emotional processing in Huntington's disease: from pre-manifest to late stage disease. Neuropsychologia (2015) 0.79
Scanning Patterns of Faces do not Explain Impaired Emotion Recognition in Huntington Disease: Evidence for a High Level Mechanism. Front Psychol (2012) 0.79
Intra-individual Variability in Prodromal Huntington Disease and Its Relationship to Genetic Burden. J Int Neuropsychol Soc (2015) 0.78
Facial emotion recognition impairments are associated with brain volume abnormalities in individuals with HIV. Neuropsychologia (2015) 0.78
Interaction without intent: the shape of the social world in Huntington's disease. Soc Cogn Affect Neurosci (2015) 0.78
Understanding the Outcomes Measures used in Huntington Disease Pharmacological Trials: A Systematic Review. J Huntingtons Dis (2014) 0.77
Evaluation of emotion processing in HIV-infected patients and correlation with cognitive performance. BMC Psychol (2013) 0.76
Pain in Neurodegenerative Disease: Current Knowledge and Future Perspectives. Behav Neurol (2016) 0.75
Laser Evoked Potentials in Early and Presymptomatic Huntington's Disease. Behav Neurol (2016) 0.75
Abnormal Visual Scanning of Emotionally Evocative Natural Scenes in Huntington's Disease. Front Psychol (2017) 0.75
Emotion recognition in frontotemporal dementia and Alzheimer's disease: A new film-based assessment. Emotion (2015) 0.75
Theory of Mind Is Impaired in Mild to Moderate Huntington's Disease Independently from Global Cognitive Functioning. Front Psychol (2017) 0.75
Parkinson's disease-associated mutations in leucine-rich repeat kinase 2 augment kinase activity. Proc Natl Acad Sci U S A (2005) 6.98
Phase 3 trials of solanezumab for mild-to-moderate Alzheimer's disease. N Engl J Med (2014) 6.67
Mutations in HFE2 cause iron overload in chromosome 1q-linked juvenile hemochromatosis. Nat Genet (2003) 5.82
Levodopa and the progression of Parkinson's disease. N Engl J Med (2004) 5.44
Early surgical therapy for drug-resistant temporal lobe epilepsy: a randomized trial. JAMA (2012) 5.40
Kinase activity of mutant LRRK2 mediates neuronal toxicity. Nat Neurosci (2006) 5.32
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat Genet (2003) 4.76
Effects of coenzyme Q10 in early Parkinson disease: evidence of slowing of the functional decline. Arch Neurol (2002) 4.60
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell (2006) 4.53
HIV-associated cognitive impairment before and after the advent of combination therapy. J Neurovirol (2002) 4.20
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci (2002) 4.15
A phase 3 trial of semagacestat for treatment of Alzheimer's disease. N Engl J Med (2013) 4.11
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet (2003) 4.07
Parkin mediates nonclassical, proteasomal-independent ubiquitination of synphilin-1: implications for Lewy body formation. J Neurosci (2005) 3.92
A schizophrenia-associated mutation of DISC1 perturbs cerebral cortex development. Nat Cell Biol (2005) 3.89
Gene delivery of AAV2-neurturin for Parkinson's disease: a double-blind, randomised, controlled trial. Lancet Neurol (2010) 3.84
Genetic variants in TPMT and COMT are associated with hearing loss in children receiving cisplatin chemotherapy. Nat Genet (2009) 3.83
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
Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc Natl Acad Sci U S A (2004) 3.73
Preparing for preventive clinical trials: the Predict-HD study. Arch Neurol (2006) 3.57
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
Beta-cell ABCA1 influences insulin secretion, glucose homeostasis and response to thiazolidinedione treatment. Nat Med (2007) 3.53
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
Disrupted-in-Schizophrenia-1 (DISC-1): mutant truncation prevents binding to NudE-like (NUDEL) and inhibits neurite outgrowth. Proc Natl Acad Sci U S A (2002) 3.34
Targeted inactivation of hepatic Abca1 causes profound hypoalphalipoproteinemia and kidney hypercatabolism of apoA-I. J Clin Invest (2005) 3.25
Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron (2003) 3.25
Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron (2002) 3.15
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease. Neuron (2005) 3.13
Huntingtin-interacting protein HIP14 is a palmitoyl transferase involved in palmitoylation and trafficking of multiple neuronal proteins. Neuron (2004) 3.04
Leucine-rich repeat kinase 2 (LRRK2) interacts with parkin, and mutant LRRK2 induces neuronal degeneration. Proc Natl Acad Sci U S A (2005) 3.00
Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data. Lancet Neurol (2011) 2.99
Differential modulation of endotoxin responsiveness by human caspase-12 polymorphisms. Nature (2004) 2.98
Mutant frizzled-4 disrupts retinal angiogenesis in familial exudative vitreoretinopathy. Nat Genet (2002) 2.90
Levodopa in the treatment of Parkinson's disease: current controversies. Mov Disord (2004) 2.90
Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity. Nat Med (2011) 2.87
Central nervous system depression of neonates breastfed by mothers receiving oxycodone for postpartum analgesia. J Pediatr (2011) 2.86
Gabapentin's effects on hot flashes in postmenopausal women: a randomized controlled trial. Obstet Gynecol (2003) 2.81
Serum urate as a predictor of clinical and radiographic progression in Parkinson disease. Arch Neurol (2008) 2.79
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Psychiatric symptoms in Huntington's disease before diagnosis: the predict-HD study. Biol Psychiatry (2007) 2.71
Initiating levodopa/carbidopa therapy with and without entacapone in early Parkinson disease: the STRIDE-PD study. Ann Neurol (2010) 2.67
Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin. Nat Med (2009) 2.66
Lipoprotein lipase S447X: a naturally occurring gain-of-function mutation. Arterioscler Thromb Vasc Biol (2006) 2.55
Intestinal ABCA1 directly contributes to HDL biogenesis in vivo. J Clin Invest (2006) 2.55
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
Psychopathology in patients with degenerative cerebellar diseases: a comparison to Huntington's disease. Am J Psychiatry (2002) 2.52
Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat Cell Biol (2002) 2.50
Urate as a predictor of the rate of clinical decline in Parkinson disease. Arch Neurol (2009) 2.46
Characterization of the Huntington intermediate CAG repeat expansion phenotype in PHAROS. Neurology (2013) 2.45
Capacity to provide informed consent for participation in schizophrenia and HIV research. Am J Psychiatry (2002) 2.41
Association between increased arterial-wall thickness and impairment in ABCA1-driven cholesterol efflux: an observational study. Lancet (2002) 2.39
Accurate prediction of the functional significance of single nucleotide polymorphisms and mutations in the ABCA1 gene. PLoS Genet (2005) 2.38
Complete rescue of lipoprotein lipase-deficient mice by somatic gene transfer of the naturally occurring LPLS447X beneficial mutation. Arterioscler Thromb Vasc Biol (2005) 2.36
Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice. Neuron (2010) 2.36
A contribution of cognitive decision models to clinical assessment: decomposing performance on the Bechara gambling task. Psychol Assess (2002) 2.32
Abnormal brain structure in children with isolated clefts of the lip or palate. Arch Pediatr Adolesc Med (2007) 2.29
Relationship between stearoyl-CoA desaturase activity and plasma triglycerides in human and mouse hypertriglyceridemia. J Lipid Res (2002) 2.28
Using cognitive models to map relations between neuropsychological disorders and human decision-making deficits. Psychol Sci (2005) 2.25
What is the risk of sham surgery in Parkinson disease clinical trials? A review of published reports. Neurology (2005) 2.25
Mutant DNA-binding domain of HSF4 is associated with autosomal dominant lamellar and Marner cataract. Nat Genet (2002) 2.21
Variations on a gene: rare and common variants in ABCA1 and their impact on HDL cholesterol levels and atherosclerosis. Annu Rev Nutr (2006) 2.20
Palmitoylation of huntingtin by HIP14 is essential for its trafficking and function. Nat Neurosci (2006) 2.19
Dopamine transporter imaging is associated with long-term outcomes in Parkinson's disease. Mov Disord (2012) 2.18
IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. J Cell Biol (2009) 2.16
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease. Proc Natl Acad Sci U S A (2005) 2.15
Neurocognitive signs in prodromal Huntington disease. Neuropsychology (2011) 2.14
Increased ABCA1 activity protects against atherosclerosis. J Clin Invest (2002) 2.13
Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization. J Biol Chem (2002) 2.12