Published in Cell on March 26, 1993
Coenzyme Q10 in Huntington's Disease (HD) (2CARE) | NCT00608881
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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
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Evidence for assembly of prions with left-handed beta-helices into trimers. Proc Natl Acad Sci U S A (2004) 3.64
The bifunctional microRNA miR-9/miR-9* regulates REST and CoREST and is downregulated in Huntington's disease. J Neurosci (2008) 3.62
The role of inflammation in the pathogenesis of age-related macular degeneration. Surv Ophthalmol (2006) 3.60
Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. J Neurosci (2008) 3.54
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Cell biology. A unifying role for prions in neurodegenerative diseases. Science (2012) 3.34
Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells. Proc Natl Acad Sci U S A (1999) 3.33
Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron (2003) 3.25
Designing siRNA that distinguish between genes that differ by a single nucleotide. PLoS Genet (2006) 3.23
CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion. Neurology (2012) 3.12
Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits. Proc Natl Acad Sci U S A (2007) 3.07
Brain-derived neurotrophic factor in neurodegenerative diseases. Nat Rev Neurol (2009) 2.93
Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression. Cell (2012) 2.83
Motor disorder in Huntington's disease begins as a dysfunction in error feedback control. Nature (2000) 2.81
Bayesian fine-scale mapping of disease loci, by hidden Markov models. Am J Hum Genet (2000) 2.72
Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin. Nat Med (2009) 2.66
HEAT repeats associated with condensins, cohesins, and other complexes involved in chromosome-related functions. Genome Res (2000) 2.66
Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes. Cell Stem Cell (2012) 2.55
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Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice. Mol Ther (2005) 2.47
Characterization of the Huntington intermediate CAG repeat expansion phenotype in PHAROS. Neurology (2013) 2.45
Assessing whether an allele can account in part for a linkage signal: the Genotype-IBD Sharing Test (GIST). Am J Hum Genet (2004) 2.43
Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice. Neuron (2009) 2.43
'Cold SSCP': a simple, rapid and non-radioactive method for optimized single-strand conformation polymorphism analyses. Nucleic Acids Res (1993) 2.38
The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice. Proc Natl Acad Sci U S A (2008) 2.37
Up-regulation of GLT1 expression increases glutamate uptake and attenuates the Huntington's disease phenotype in the R6/2 mouse. Neuroscience (2008) 2.31
CAG-repeat length and the age of onset in Huntington disease (HD): a review and validation study of statistical approaches. Am J Med Genet B Neuropsychiatr Genet (2010) 2.31
Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models. Neurobiol Dis (2009) 2.29
Prion-like disorders: blurring the divide between transmissibility and infectivity. J Cell Sci (2010) 2.29
A genomic screen in yeast implicates kynurenine 3-monooxygenase as a therapeutic target for Huntington disease. Nat Genet (2005) 2.28
Linkage disequilibrium and allele-frequency distributions for 114 single-nucleotide polymorphisms in five populations. Am J Hum Genet (2000) 2.25
Identifying polyglutamine protein species in situ that best predict neurodegeneration. Nat Chem Biol (2011) 2.24
Does neuroinflammation fan the flame in neurodegenerative diseases? Mol Neurodegener (2009) 2.23
Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients. Curr Biol (2009) 2.19
Palmitoylation of huntingtin by HIP14 is essential for its trafficking and function. Nat Neurosci (2006) 2.19
Interaction of Huntington disease protein with transcriptional activator Sp1. Mol Cell Biol (2002) 2.19
N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking. J Neurosci (2008) 2.18
The kynurenine pathway modulates neurodegeneration in a Drosophila model of Huntington's disease. Curr Biol (2011) 2.17
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease. Proc Natl Acad Sci U S A (2005) 2.15
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
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Huntingtin-associated protein 1 regulates inhibitory synaptic transmission by modulating gamma-aminobutyric acid type A receptor membrane trafficking. Proc Natl Acad Sci U S A (2004) 2.10
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SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis. Proc Natl Acad Sci U S A (2010) 2.09
The genetic epidemiology of neurodegenerative disease. J Clin Invest (2005) 2.08
PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function. Sci Transl Med (2012) 2.07
Neuroimaging distinction between neurological and psychiatric disorders†. Br J Psychiatry (2015) 2.03
Factors affecting statistical power in the detection of genetic association. J Clin Invest (2005) 2.02
Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease. Proc Natl Acad Sci U S A (2004) 2.00
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
Effects of overexpression of huntingtin proteins on mitochondrial integrity. Hum Mol Genet (2008) 1.98
Secondary structure of Huntingtin amino-terminal region. Structure (2009) 1.98
Effect of trehalose on protein structure. Protein Sci (2009) 1.97
Oxidative stress, mitochondrial dysfunction, and aging. J Signal Transduct (2011) 1.94
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Protection from mitochondrial complex II inhibition in vitro and in vivo by Nrf2-mediated transcription. Proc Natl Acad Sci U S A (2004) 1.89
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
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Factors associated with HD CAG repeat instability in Huntington disease. J Med Genet (2007) 1.86
Transcriptional signatures in Huntington's disease. Prog Neurobiol (2007) 1.86
A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study. Am J Hum Genet (2003) 1.85
Basal Ganglia disorders associated with imbalances in the striatal striosome and matrix compartments. Front Neuroanat (2011) 1.85
A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila. Proc Natl Acad Sci U S A (2003) 1.84
Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron. Proc Natl Acad Sci U S A (1999) 1.84
Inhibition of mitochondrial fragmentation diminishes Huntington's disease-associated neurodegeneration. J Clin Invest (2013) 1.83
Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin. Mol Ther (2011) 1.82
Striatal and white matter predictors of estimated diagnosis for Huntington disease. Brain Res Bull (2010) 1.79
Mutant CAG repeats of Huntingtin transcript fold into hairpins, form nuclear foci and are targets for RNA interference. Nucleic Acids Res (2011) 1.77
Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice. Neuroscience (2008) 1.76
Histone deacetylase inhibitors reduce polyglutamine toxicity. Proc Natl Acad Sci U S A (2001) 1.74
Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. J Clin Invest (2015) 1.74
Motor abnormalities in premanifest persons with Huntington's disease: the PREDICT-HD study. Mov Disord (2009) 1.73
Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease. J Neurosci (2009) 1.73
Elimination of prions by branched polyamines and implications for therapeutics. Proc Natl Acad Sci U S A (1999) 1.73
A systematic strategy for large-scale analysis of genotype phenotype correlations: identification of candidate genes involved in African trypanosomiasis. Nucleic Acids Res (2007) 1.73
Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli. EMBO Mol Med (2010) 1.72
Impaired TrkB receptor signaling underlies corticostriatal dysfunction in Huntington's disease. Neuron (2014) 1.72
Preclinical safety of RNAi-mediated HTT suppression in the rhesus macaque as a potential therapy for Huntington's disease. Mol Ther (2011) 1.72
Huntington's disease: can mice lead the way to treatment? Neuron (2011) 1.71
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
Identification of NUB1 as a suppressor of mutant Huntington toxicity via enhanced protein clearance. Nat Neurosci (2013) 1.71
Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease. Nat Med (2014) 1.67