Published in J Neurochem on November 06, 2007
Polyglutamine neurodegeneration: protein misfolding revisited. Trends Neurosci (2008) 2.63
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
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
Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo. J Biol Chem (2010) 1.62
Suppression of protein aggregation by chaperone modification of high molecular weight complexes. Brain (2012) 1.42
Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein. Nat Biotechnol (2010) 1.38
Examining polyglutamine peptide length: a connection between collapsed conformations and increased aggregation. J Mol Biol (2009) 1.36
Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice. PLoS Genet (2010) 1.30
In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis. Neurobiol Dis (2008) 1.28
Tracking mutant huntingtin aggregation kinetics in cells reveals three major populations that include an invariant oligomer pool. J Biol Chem (2010) 1.28
Optimisation of region-specific reference gene selection and relative gene expression analysis methods for pre-clinical trials of Huntington's disease. Mol Neurodegener (2008) 1.20
CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse. Neurobiol Dis (2008) 1.19
The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease. Mol Neurodegener (2010) 1.18
Mouse models of Huntington's disease and methodological considerations for therapeutic trials. Biochim Biophys Acta (2009) 1.14
Heterologous expression and extracellular secretion of cellulolytic enzymes by Zymomonas mobilis. Appl Environ Microbiol (2010) 1.08
Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein. J Biol Chem (2012) 1.07
Huntington's disease: progress toward effective disease-modifying treatments and a cure. Hum Mol Genet (2010) 1.05
Dendritic spine loss and neurodegeneration is rescued by Rab11 in models of Huntington's disease. Cell Death Differ (2010) 1.04
Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias. Cerebellum (2014) 1.03
Conformational targeting of fibrillar polyglutamine proteins in live cells escalates aggregation and cytotoxicity. PLoS One (2009) 1.01
Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington's disease. Neurobiol Dis (2010) 0.99
Bifunctional anti-huntingtin proteasome-directed intrabodies mediate efficient degradation of mutant huntingtin exon 1 protein fragments. PLoS One (2011) 0.93
Exogenous delivery of chaperonin subunit fragment ApiCCT1 modulates mutant Huntingtin cellular phenotypes. Proc Natl Acad Sci U S A (2013) 0.93
Methylene blue modulates huntingtin aggregation intermediates and is protective in Huntington's disease models. J Neurosci (2012) 0.92
Inducible mutant huntingtin expression in HN10 cells reproduces Huntington's disease-like neuronal dysfunction. Mol Neurodegener (2009) 0.91
Trinucleotide repeats: a structural perspective. Front Neurol (2013) 0.89
Detection of Mutant Huntingtin Aggregation Conformers and Modulation of SDS-Soluble Fibrillar Oligomers by Small Molecules. J Huntingtons Dis (2012) 0.89
An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity. Cell Death Dis (2012) 0.88
Aggregation of polyQ proteins is increased upon yeast aging and affected by Sir2 and Hsf1: novel quantitative biochemical and microscopic assays. PLoS One (2012) 0.87
Aggregation formation in the polyglutamine diseases: protection at a cost? Mol Cells (2013) 0.87
Blocking the association of HDAC4 with MAP1S accelerates autophagy clearance of mutant Huntingtin. Aging (Albany NY) (2015) 0.84
Monomeric, oligomeric and polymeric proteins in huntington disease and other diseases of polyglutamine expansion. Brain Sci (2014) 0.84
Post-aggregation oxidation of mutant huntingtin controls the interactions between aggregates. J Biol Chem (2012) 0.83
From pathways to targets: understanding the mechanisms behind polyglutamine disease. Biomed Res Int (2014) 0.81
Mutant huntingtin gene-dose impacts on aggregate deposition, DARPP32 expression and neuroinflammation in HdhQ150 mice. PLoS One (2013) 0.81
Proteasome-mediated proteolysis of the polyglutamine-expanded androgen receptor is a late event in spinal and bulbar muscular atrophy (SBMA) pathogenesis. J Biol Chem (2015) 0.80
The Aggregation of Huntingtin and α-Synuclein. J Biophys (2012) 0.80
Folding Landscape of Mutant Huntingtin Exon1: Diffusible Multimers, Oligomers and Fibrils, and No Detectable Monomer. PLoS One (2016) 0.80
Identification of novel polyglutamine-expanded aggregation species in spinal and bulbar muscular atrophy. Brain Res (2015) 0.78
p62 plays a protective role in the autophagic degradation of polyglutamine protein oligomers in polyglutamine disease model flies. J Biol Chem (2014) 0.78
Studying polyglutamine aggregation in Caenorhabditis elegans using an analytical ultracentrifuge equipped with fluorescence detection. Protein Sci (2015) 0.77
Control of the structural landscape and neuronal proteotoxicity of mutant Huntingtin by domains flanking the polyQ tract. Elife (2016) 0.77
Atg4b-dependent autophagic flux alleviates Huntington's disease progression. PLoS One (2013) 0.77
Experimental models for identifying modifiers of polyglutamine-induced aggregation and neurodegeneration. Neurotherapeutics (2013) 0.76
FUS/TLS acts as an aggregation-dependent modifier of polyglutamine disease model mice. Sci Rep (2016) 0.76
The Guanine nucleotide exchange factor kalirin-7 is a novel synphilin-1 interacting protein and modifies synphilin-1 aggregate transport and formation. PLoS One (2012) 0.76
Bifunctional Anti-Non-Amyloid Component α-Synuclein Nanobodies Are Protective In Situ. PLoS One (2016) 0.75
Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 mice. PLoS One (2013) 0.75
Sedimentation Velocity Analysis With Fluorescence Detection of Mutant Huntingtin Exon 1 Aggregation in Drosophila Melanogaster and Caenorhabditis Elegans. Biochemistry (2017) 0.75
Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy (2012) 20.08
Lineage-specific polycomb targets and de novo DNA methylation define restriction and potential of neuronal progenitors. Mol Cell (2008) 7.68
Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host. Science (2006) 6.47
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
Identification of a population of blood circulating tumor cells from breast cancer patients that initiates metastasis in a xenograft assay. Nat Biotechnol (2013) 4.11
Global changes to the ubiquitin system in Huntington's disease. Nature (2007) 3.91
Radial glia serve as neuronal progenitors in all regions of the central nervous system. Neuron (2004) 3.58
Characterizing light-regulated retinal microRNAs reveals rapid turnover as a common property of neuronal microRNAs. Cell (2010) 3.57
Role of EDEM in the release of misfolded glycoproteins from the calnexin cycle. Science (2003) 3.48
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Mass-spectrometric identification and relative quantification of N-linked cell surface glycoproteins. Nat Biotechnol (2009) 2.70
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
Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series. EMBO Rep (2004) 2.35
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
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
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
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
Environmental enrichment slows disease progression in R6/2 Huntington's disease mice. Ann Neurol (2002) 1.74
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
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
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
Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. J Proteome Res (2007) 1.59
Neurotrophin receptors TrkA and TrkC cause neuronal death whereas TrkB does not. Nature (2010) 1.56
Gene expression in cortical interneuron precursors is prescient of their mature function. Cereb Cortex (2008) 1.51
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
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
Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease. Hum Mol Genet (2005) 1.39
Hsp104 antagonizes alpha-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease. J Clin Invest (2008) 1.38
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
Targeting presenilin-type aspartic protease signal peptide peptidase with gamma-secretase inhibitors. J Biol Chem (2003) 1.33
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
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
Akt is altered in an animal model of Huntington's disease and in patients. Eur J Neurosci (2005) 1.27
Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease. Mol Ther (2007) 1.26
Identification of a lectin causing the degeneration of neuronal processes using engineered embryonic stem cells. Nat Neurosci (2007) 1.25
Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry. J Neurosci (2008) 1.23
Optimisation of region-specific reference gene selection and relative gene expression analysis methods for pre-clinical trials of Huntington's disease. Mol Neurodegener (2008) 1.20
The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease. Mol Neurodegener (2010) 1.18
A brain-permeable small molecule reduces neuronal cholesterol by inhibiting activity of sirtuin 2 deacetylase. ACS Chem Biol (2011) 1.17
Inhibition of calcineurin by FK506 protects against polyglutamine-huntingtin toxicity through an increase of huntingtin phosphorylation at S421. J Neurosci (2006) 1.16
Genetic knock-down of HDAC7 does not ameliorate disease pathogenesis in the R6/2 mouse model of Huntington's disease. PLoS One (2009) 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
Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression. J Clin Invest (2012) 1.14
Expression of human beta-secretase in the mouse brain increases the steady-state level of beta-amyloid. J Neurochem (2002) 1.14
HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation. Brain (2014) 1.13
TR-FRET-based duplex immunoassay reveals an inverse correlation of soluble and aggregated mutant huntingtin in huntington's disease. Chem Biol (2012) 1.13
The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease. J Clin Invest (2011) 1.11
Increased metabolism in the R6/2 mouse model of Huntington's disease. Neurobiol Dis (2007) 1.11
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
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
Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression. Hum Mol Genet (2010) 1.06
Detection of a soluble form of BACE-1 in human cerebrospinal fluid by a sensitive activity assay. Clin Chem (2006) 1.05
Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice. Neurobiol Dis (2006) 1.03