Published in J Biol Chem on January 19, 2010
Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease. Proc Natl Acad Sci U S A (2013) 2.21
HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration. PLoS Biol (2013) 1.96
Identification of NUB1 as a suppressor of mutant Huntington toxicity via enhanced protein clearance. Nat Neurosci (2013) 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- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopy. PLoS One (2014) 1.48
Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease. Neuron (2010) 1.45
Suppression of protein aggregation by chaperone modification of high molecular weight complexes. Brain (2012) 1.42
SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice. PLoS One (2016) 1.41
Unmasking the roles of N- and C-terminal flanking sequences from exon 1 of huntingtin as modulators of polyglutamine aggregation. Proc Natl Acad Sci U S A (2013) 1.39
Huntington's disease: underlying molecular mechanisms and emerging concepts. Trends Biochem Sci (2013) 1.31
SIRT2 ablation has no effect on tubulin acetylation in brain, cholesterol biosynthesis or the progression of Huntington's disease phenotypes in vivo. PLoS One (2012) 1.27
Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression. J Clin Invest (2012) 1.14
Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments. Hum Mol Genet (2010) 1.08
Small changes, big impact: posttranslational modifications and function of huntingtin in Huntington disease. Neuroscientist (2011) 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
In vitro and in vivo aggregation of a fragment of huntingtin protein directly causes free radical production. J Biol Chem (2011) 1.05
Huntington's disease: progress toward effective disease-modifying treatments and a cure. Hum Mol Genet (2010) 1.05
Wild-type HTT modulates the enzymatic activity of the neuronal palmitoyl transferase HIP14. Hum Mol Genet (2011) 1.05
A review of quality of life after predictive testing for and earlier identification of neurodegenerative diseases. Prog Neurobiol (2013) 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
An N-terminal nuclear export signal regulates trafficking and aggregation of Huntingtin (Htt) protein exon 1. J Biol Chem (2013) 0.99
Translation of HTT mRNA with expanded CAG repeats is regulated by the MID1-PP2A protein complex. Nat Commun (2013) 0.99
Huntington's disease and its therapeutic target genes: a global functional profile based on the HD Research Crossroads database. BMC Neurol (2012) 0.96
Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation. EMBO J (2015) 0.95
The interaction of polyglutamine peptides with lipid membranes is regulated by flanking sequences associated with huntingtin. J Biol Chem (2013) 0.94
HDAC4 does not act as a protein deacetylase in the postnatal murine brain in vivo. PLoS One (2013) 0.94
Bifunctional anti-huntingtin proteasome-directed intrabodies mediate efficient degradation of mutant huntingtin exon 1 protein fragments. PLoS One (2011) 0.93
PolyQ disease: misfiring of a developmental cell death program? Trends Cell Biol (2012) 0.93
Cysteine oxidation within N-terminal mutant huntingtin promotes oligomerization and delays clearance of soluble protein. J Biol Chem (2011) 0.93
Transgenic mouse model expressing the caspase 6 fragment of mutant huntingtin. J Neurosci (2012) 0.92
Differential ubiquitination and degradation of huntingtin fragments modulated by ubiquitin-protein ligase E3A. Proc Natl Acad Sci U S A (2014) 0.91
Disruption of the nuclear membrane by perinuclear inclusions of mutant huntingtin causes cell-cycle re-entry and striatal cell death in mouse and cell models of Huntington's disease. Hum Mol Genet (2014) 0.90
Impaired adult olfactory bulb neurogenesis in the R6/2 mouse model of Huntington's disease. BMC Neurosci (2010) 0.90
Calpain inhibition mediates autophagy-dependent protection against polyglutamine toxicity. Cell Death Differ (2014) 0.90
Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis. FEBS J (2013) 0.90
Cysteine proteases bleomycin hydrolase and cathepsin Z mediate N-terminal proteolysis and toxicity of mutant huntingtin. J Biol Chem (2011) 0.90
Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/2 mouse model of HD. PLoS One (2013) 0.89
Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment. Hum Mol Genet (2011) 0.89
SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation. Cell Rep (2013) 0.88
The role of IκB kinase complex in the neurobiology of Huntington's disease. Neurobiol Dis (2011) 0.87
Aggregation behavior of chemically synthesized, full-length huntingtin exon1. Biochemistry (2014) 0.87
Synaptic mutant huntingtin inhibits synapsin-1 phosphorylation and causes neurological symptoms. J Cell Biol (2013) 0.86
Mouse models of polyglutamine diseases in therapeutic approaches: review and data table. Part II. Mol Neurobiol (2012) 0.86
Transgenic animal models for study of the pathogenesis of Huntington's disease and therapy. Drug Des Devel Ther (2015) 0.86
Mood disorders in Huntington's disease: from behavior to cellular and molecular mechanisms. Front Behav Neurosci (2014) 0.86
Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors. Mol Neurodegener (2010) 0.85
Comparative study of naturally occurring huntingtin fragments in Drosophila points to exon 1 as the most pathogenic species in Huntington's disease. Hum Mol Genet (2014) 0.84
A coarse-grained model for polyglutamine aggregation modulated by amphipathic flanking sequences. Biophys J (2014) 0.84
Temporal separation of aggregation and ubiquitination during early inclusion formation in transgenic mice carrying the Huntington's disease mutation. PLoS One (2012) 0.84
Monomeric, oligomeric and polymeric proteins in huntington disease and other diseases of polyglutamine expansion. Brain Sci (2014) 0.84
Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer. J Biol Chem (2012) 0.84
N17 Modifies mutant Huntingtin nuclear pathogenesis and severity of disease in HD BAC transgenic mice. Neuron (2015) 0.83
Modulation of mutant huntingtin N-terminal cleavage and its effect on aggregation and cell death. Neurotox Res (2010) 0.83
Aberrantly spliced HTT, a new player in Huntington's disease pathogenesis. RNA Biol (2013) 0.83
RAN translation and frameshifting as translational challenges at simple repeats of human neurodegenerative disorders. Nucleic Acids Res (2014) 0.83
TR-FRET assays of Huntingtin protein fragments reveal temperature and polyQ length-dependent conformational changes. Sci Rep (2014) 0.83
Huntingtin localisation studies - a technical review. PLoS Curr (2011) 0.82
Contesting the dogma of an age-related heat shock response impairment: implications for cardiac-specific age-related disorders. Hum Mol Genet (2014) 0.82
Identification of anti-inflammatory targets for Huntington's disease using a brain slice-based screening assay. Neurobiol Dis (2011) 0.82
Perturbation with intrabodies reveals that calpain cleavage is required for degradation of huntingtin exon 1. PLoS One (2011) 0.82
From pathways to targets: understanding the mechanisms behind polyglutamine disease. Biomed Res Int (2014) 0.81
Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington's disease. PLoS Curr (2012) 0.81
Mutant huntingtin gene-dose impacts on aggregate deposition, DARPP32 expression and neuroinflammation in HdhQ150 mice. PLoS One (2013) 0.81
Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon aging. PLoS One (2012) 0.81
Architecture of polyglutamine-containing fibrils from time-resolved fluorescence decay. J Biol Chem (2014) 0.81
Analysis of proteolytic processes and enzymatic activities in the generation of huntingtin n-terminal fragments in an HEK293 cell model. PLoS One (2012) 0.81
Characterization of Striatal Neuronal Loss and Atrophy in the R6/2 Mouse Model of Huntington's Disease. PLoS Curr (2014) 0.80
Integration-independent Transgenic Huntington Disease Fragment Mouse Models Reveal Distinct Phenotypes and Life Span in Vivo. J Biol Chem (2015) 0.79
Inhibition of DNA Methyltransferases Blocks Mutant Huntingtin-Induced Neurotoxicity. Sci Rep (2016) 0.79
Pathogenic polyglutamine expansion length correlates with polarity of the flanking sequences. Mol Neurodegener (2014) 0.79
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
In Vivo Profiling Reveals a Competent Heat Shock Response in Adult Neurons: Implications for Neurodegenerative Disorders. PLoS One (2015) 0.78
Comparing ESC and iPSC-Based Models for Human Genetic Disorders. J Clin Med (2014) 0.78
Ubiquitin-activating enzyme activity contributes to differential accumulation of mutant huntingtin in brain and peripheral tissues. J Neurosci (2014) 0.78
Primary cilia and autophagic dysfunction in Huntington's disease. Cell Death Differ (2015) 0.78
Selection and characterization of llama single domain antibodies against N-terminal huntingtin. Neurol Sci (2014) 0.78
Control of the structural landscape and neuronal proteotoxicity of mutant Huntingtin by domains flanking the polyQ tract. Elife (2016) 0.77
A protein polymerization cascade mediates toxicity of non-pathological human huntingtin in yeast. Sci Rep (2015) 0.77
FUS/TLS acts as an aggregation-dependent modifier of polyglutamine disease model mice. Sci Rep (2016) 0.76
VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease. Nat Commun (2016) 0.76
The emerging role of the first 17 amino acids of huntingtin in Huntington's disease. Biomol Concepts (2015) 0.76
Acetylation within the First 17 Residues of Huntingtin Exon 1 Alters Aggregation and Lipid Binding. Biophys J (2016) 0.76
The N17 domain mitigates nuclear toxicity in a novel zebrafish Huntington's disease model. Mol Neurodegener (2015) 0.75
Cholesterol Modifies Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes. Biochemistry (2015) 0.75
Similar striatal gene expression profiles in the striatum of the YAC128 and HdhQ150 mouse models of Huntington's disease are not reflected in mutant Huntingtin inclusion prevalence. BMC Genomics (2015) 0.75
Calpain-5 Expression in the Retina Localizes to Photoreceptor Synapses. Invest Ophthalmol Vis Sci (2016) 0.75
A new Caenorhabditis elegans model of human huntingtin 513 aggregation and toxicity in body wall muscles. PLoS One (2017) 0.75
Comparison of mHTT Antibodies in Huntington's Disease Mouse Models Reveal Specific Binding Profiles and Steady-State Ubiquitin Levels with Disease Development. PLoS One (2016) 0.75
Polyglutamine expansion affects huntingtin conformation in multiple Huntington's disease models. Sci Rep (2017) 0.75
Disruption to schizophrenia-associated gene Fez1 in the hippocampus of HDAC11 knockout mice. Sci Rep (2017) 0.75
HSF1-dependent and -independent regulation of the mammalian in vivo heat shock response and its impairment in Huntington's disease mouse models. Sci Rep (2017) 0.75
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Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science (1997) 11.53
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell (1997) 7.59
Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases. Proc Natl Acad Sci U S A (1994) 6.96
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell (2006) 4.53
Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum Mol Genet (2001) 3.84
The hunt for huntingtin function: interaction partners tell many different stories. Trends Biochem Sci (2003) 3.15
Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nat Genet (1996) 3.08
Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions. Mol Cell (2002) 2.98
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum Mol Genet (2000) 2.67
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
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat Genet (1998) 2.42
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Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. J Neurosci (2002) 2.05
New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins. Brain Res Bull (2001) 2.03
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
Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis. Proc Natl Acad Sci U S A (2001) 1.99
Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. Hum Mol Genet (1999) 1.86
Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus. J Biol Chem (2004) 1.82
Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity. J Cell Biol (2003) 1.77
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
Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. Hum Mol Genet (2008) 1.70
Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease. Anal Biochem (2009) 1.64
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
Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells. J Biol Chem (2009) 1.47
Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease. Hum Mol Genet (2005) 1.39
Reversal of a full-length mutant huntingtin neuronal cell phenotype by chemical inhibitors of polyglutamine-mediated aggregation. BMC Neurosci (2005) 1.35
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
N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease. Cell Cycle (2007) 1.31
Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus. Hum Mol Genet (2008) 1.26
Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models. J Neuropathol Exp Neurol (2007) 1.25
Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles. Neurobiol Dis (2001) 1.23
Huntingtin contains a highly conserved nuclear export signal. Hum Mol Genet (2003) 1.15
Imaging polyglutamine deposits in brain tissue. Methods Enzymol (2006) 1.13
Detection of polyglutamine aggregation in mouse models. Methods Enzymol (1999) 1.09
Lysosomal proteases are involved in generation of N-terminal huntingtin fragments. Neurobiol Dis (2006) 1.08
Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant huntingtin. Neurobiol Dis (2005) 1.01
Polyglutamine repeat length-dependent proteolysis of huntingtin. Neurobiol Dis (2002) 1.00
Development of a method for the high-throughput quantification of cellular proteins. Chembiochem (2009) 0.93
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EML4-ALK fusion gene and efficacy of an ALK kinase inhibitor in lung cancer. Clin Cancer Res (2008) 9.29
Dickkopf-1 is a master regulator of joint remodeling. Nat Med (2007) 7.29
Homozygous deletions and chromosome amplifications in human lung carcinomas revealed by single nucleotide polymorphism array analysis. Cancer Res (2005) 6.93
Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host. Science (2006) 6.47
Leptin regulation of bone resorption by the sympathetic nervous system and CART. Nature (2005) 6.43
Transcriptome sequencing of malignant pleural mesothelioma tumors. Proc Natl Acad Sci U S A (2008) 4.75
Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis. Neuron (2012) 4.48
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
Translation of microarray data into clinically relevant cancer diagnostic tests using gene expression ratios in lung cancer and mesothelioma. Cancer Res (2002) 3.90
Role of EDEM in the release of misfolded glycoproteins from the calnexin cycle. Science (2003) 3.48
Activating mutations of the noonan syndrome-associated SHP2/PTPN11 gene in human solid tumors and adult acute myelogenous leukemia. Cancer Res (2004) 3.20
Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions. Mol Cell (2002) 2.98
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Huntingtin interacting proteins are genetic modifiers of neurodegeneration. PLoS Genet (2007) 2.77
Coupling endoplasmic reticulum stress to the cell death program: role of the ER chaperone GRP78. FEBS Lett (2002) 2.71
Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation. Proc Natl Acad Sci U S A (2005) 2.69
Dkk1-mediated inhibition of Wnt signaling in bone results in osteopenia. Bone (2006) 2.59
Acetylation targets mutant huntingtin to autophagosomes for degradation. Cell (2009) 2.57
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
Androgen receptor YAC transgenic mice recapitulate SBMA motor neuronopathy and implicate VEGF164 in the motor neuron degeneration. Neuron (2004) 2.52
Missense mutations of the BRAF gene in human lung adenocarcinoma. Cancer Res (2002) 2.48
Gene expression in lung adenocarcinomas of smokers and nonsmokers. Am J Respir Cell Mol Biol (2003) 2.45
Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice. Neuron (2009) 2.43
FGF23 neutralization improves chronic kidney disease-associated hyperparathyroidism yet increases mortality. J Clin Invest (2012) 2.41
Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series. EMBO Rep (2004) 2.35
Identifying polyglutamine protein species in situ that best predict neurodegeneration. Nat Chem Biol (2011) 2.24
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Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease. Proc Natl Acad Sci U S A (2013) 2.21
Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction. PLoS Biol (2006) 2.12
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
Neuroglobin-overexpressing transgenic mice are resistant to cerebral and myocardial ischemia. Proc Natl Acad Sci U S A (2006) 2.05
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. J Neurosci (2002) 2.05
An antisense CAG repeat transcript at JPH3 locus mediates expanded polyglutamine protein toxicity in Huntington's disease-like 2 mice. Neuron (2011) 2.00
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
Early autophagic response in a novel knock-in model of Huntington disease. Hum Mol Genet (2010) 1.99
HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration. PLoS Biol (2013) 1.96
Phase III intergroup study of talc poudrage vs talc slurry sclerosis for malignant pleural effusion. Chest (2005) 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
Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus. J Biol Chem (2004) 1.82
Sclerostin and Dickkopf-1 as therapeutic targets in bone diseases. Endocr Rev (2012) 1.82
Genetic correction of Huntington's disease phenotypes in induced pluripotent stem cells. Cell Stem Cell (2012) 1.79
Solution structure of polyglutamine tracts in GST-polyglutamine fusion proteins. FEBS Lett (2002) 1.78
Nodal downstaging predicts survival following induction chemotherapy for stage IIIA (N2) non-small cell lung cancer in CALGB protocol #8935. J Surg Oncol (2006) 1.75
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
Loss of the Tg737 protein results in skeletal patterning defects. Dev Dyn (2003) 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
Characterization of Human Huntington's Disease Cell Model from Induced Pluripotent Stem Cells. PLoS Curr (2010) 1.70
Sequential assistance of molecular chaperones and transient formation of covalent complexes during protein degradation from the ER. J Cell Biol (2002) 1.68
Dysfunction of the cholesterol biosynthetic pathway in Huntington's disease. J Neurosci (2005) 1.68
Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease. J Clin Invest (2011) 1.66
Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease. Anal Biochem (2009) 1.64
Calpain activation in Huntington's disease. J Neurosci (2002) 1.63
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
Identification of novel candidate oncogenes and tumor suppressors in malignant pleural mesothelioma using large-scale transcriptional profiling. Am J Pathol (2005) 1.60
Using gene expression ratios to predict outcome among patients with mesothelioma. J Natl Cancer Inst (2003) 1.59
Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. J Proteome Res (2007) 1.59
Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease. J Neurochem (2007) 1.54
The calcium sensing receptor modulates fluid reabsorption and acid secretion in the proximal tubule. Kidney Int (2013) 1.49
Impaired coordination of nutrient intake and substrate oxidation in melanocortin-4 receptor knockout mice. Endocrinology (2003) 1.49
FGF-2 promotes neurogenesis and neuroprotection and prolongs survival in a transgenic mouse model of Huntington's disease. Proc Natl Acad Sci U S A (2005) 1.46
EDEM1 regulates ER-associated degradation by accelerating de-mannosylation of folding-defective polypeptides and by inhibiting their covalent aggregation. Biochem Biophys Res Commun (2006) 1.46
Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease. Neuron (2010) 1.45
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