PubRank

Henry L Paulson

Author PubWeight™ 99.80‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 siRNA-mediated gene silencing in vitro and in vivo. Nat Biotechnol 2002 5.65
2 RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model. Proc Natl Acad Sci U S A 2005 4.79
3 RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia. Nat Med 2004 4.63
4 Allele-specific silencing of dominant disease genes. Proc Natl Acad Sci U S A 2003 3.00
5 CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome. Neuron 2013 2.94
6 Polyglutamine neurodegeneration: protein misfolding revisited. Trends Neurosci 2008 2.63
7 RNA-mediated neurodegeneration in repeat expansion disorders. Ann Neurol 2010 2.30
8 The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains. J Biol Chem 2008 2.09
9 Brain structure in preclinical Huntington's disease. Biol Psychiatry 2005 2.05
10 Early autophagic response in a novel knock-in model of Huntington disease. Hum Mol Genet 2010 1.99
11 Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism. Mol Cell 2005 1.84
12 Aberrant cellular behavior of mutant torsinA implicates nuclear envelope dysfunction in DYT1 dystonia. J Neurosci 2004 1.71
13 CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J Neurosci 2005 1.57
14 Silencing primary dystonia: lentiviral-mediated RNA interference therapy for DYT1 dystonia. J Neurosci 2005 1.53
15 Spinocerebellar ataxias: an update. Curr Opin Neurol 2007 1.48
16 Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis. Proc Natl Acad Sci U S A 2002 1.48
17 Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3. J Neurosci 2011 1.46
18 Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3. EMBO J 2009 1.46
19 Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways. J Biol Chem 2003 1.44
20 Toward understanding Machado-Joseph disease. Prog Neurobiol 2011 1.42
21 Protein aggregation and the ubiquitin proteasome pathway: gaining the UPPer hand on neurodegeneration. Curr Opin Genet Dev 2003 1.41
22 Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP. Mol Cell 2011 1.37
23 Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3. J Biol Chem 2005 1.34
24 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
25 Genome-wide DNA methylation differences between late-onset Alzheimer's disease and cognitively normal controls in human frontal cortex. J Alzheimers Dis 2012 1.27
26 Targeting Alzheimer's disease genes with RNA interference: an efficient strategy for silencing mutant alleles. Nucleic Acids Res 2004 1.27
27 The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability. Hum Mol Genet 2010 1.21
28 Molecular medicine for the brain: silencing of disease genes with RNA interference. Lancet Neurol 2004 1.20
29 Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration. Hum Mol Genet 2009 1.18
30 Obsessive and compulsive symptoms in prediagnosed Huntington's disease. J Clin Psychiatry 2008 1.17
31 Balancing act: deubiquitinating enzymes in the nervous system. Trends Neurosci 2011 1.17
32 Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study. Orphanet J Rare Dis 2013 1.15
33 Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity. J Biol Chem 2007 1.14
34 Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117. J Biol Chem 2010 1.12
35 Accelerated neurodegeneration through chaperone-mediated oligomerization of tau. J Clin Invest 2013 1.12
36 Cognitive decline and aging: the role of concussive and subconcussive impacts. Exerc Sport Sci Rev 2012 1.11
37 Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome. PLoS Genet 2010 1.11
38 Allele-specific RNAi mitigates phenotypic progression in a transgenic model of Alzheimer's disease. Mol Ther 2009 1.09
39 Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3. J Neurochem 2004 1.08
40 Selective cochlear degeneration in mice lacking the F-box protein, Fbx2, a glycoprotein-specific ubiquitin ligase subunit. J Neurosci 2007 1.06
41 Diversity in tissue expression, substrate binding, and SCF complex formation for a lectin family of ubiquitin ligases. J Biol Chem 2008 1.05
42 The ubiquitin-conjugating enzyme (E2) Ube2w ubiquitinates the N terminus of substrates. J Biol Chem 2013 1.03
43 Toward therapy for DYT1 dystonia: allele-specific silencing of mutant TorsinA. Ann Neurol 2003 1.00
44 Understanding the role of the Josephin domain in the PolyUb binding and cleavage properties of ataxin-3. PLoS One 2010 0.99
45 Technology insight: therapeutic RNA interference--how far from the neurology clinic? Nat Clin Pract Neurol 2007 0.99
46 A novel route for F-box protein-mediated ubiquitination links CHIP to glycoprotein quality control. J Biol Chem 2006 0.99
47 Destabilization of a non-pathological variant of ataxin-3 results in fibrillogenesis via a partially folded intermediate: a model for misfolding in polyglutamine disease. J Mol Biol 2004 0.98
48 The E3 ubiquitin ligase CHIP and the molecular chaperone Hsc70 form a dynamic, tethered complex. Biochemistry 2013 0.95
49 Analysis of the tau-associated proteome reveals that exchange of Hsp70 for Hsp90 is involved in tau degradation. ACS Chem Biol 2012 0.94
50 Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice. Mol Ther 2013 0.94
51 Degeneration of the cerebellum in Huntington's disease (HD): possible relevance for the clinical picture and potential gateway to pathological mechanisms of the disease process. Brain Pathol 2012 0.92
52 Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease. Mol Ther 2013 0.91
53 Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties. PLoS One 2010 0.91
54 Physiologic alterations in ataxia: channeling changes into novel therapies. Arch Neurol 2009 0.90
55 Caring for Machado-Joseph disease: current understanding and how to help patients. Parkinsonism Relat Disord 2009 0.88
56 Risperidone and the treatment of psychiatric, motor, and cognitive symptoms in Huntington's disease. Ann Clin Psychiatry 2008 0.88
57 Early alterations of autophagy in Huntington disease-like mice. Autophagy 2010 0.87
58 Splice isoform-specific suppression of the Cav2.1 variant underlying spinocerebellar ataxia type 6. Neurobiol Dis 2011 0.86
59 Alzheimer's disease and environmental exposure to lead: the epidemiologic evidence and potential role of epigenetics. Curr Alzheimer Res 2012 0.86
60 JosD1, a membrane-targeted deubiquitinating enzyme, is activated by ubiquitination and regulates membrane dynamics, cell motility, and endocytosis. J Biol Chem 2013 0.85
61 RNA interference in neuroscience: progress and challenges. Cell Mol Neurobiol 2005 0.84
62 Establishment of a novel fluorescence-based method to evaluate chaperone-mediated autophagy in a single neuron. PLoS One 2012 0.83
63 F-box only protein 2 (Fbxo2) regulates amyloid precursor protein levels and processing. J Biol Chem 2014 0.83
64 Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1. Dis Model Mech 2013 0.82
65 Ataxin-3 plays a role in mouse myogenic differentiation through regulation of integrin subunit levels. PLoS One 2010 0.82
66 Compromised mitochondrial complex II in models of Machado-Joseph disease. Biochim Biophys Acta 2011 0.82
67 Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3. PLoS One 2012 0.81
68 Enzymatic production of mono-ubiquitinated proteins for structural studies: The example of the Josephin domain of ataxin-3. FEBS Open Bio 2013 0.79
69 C9orf72-associated FTD/ALS: when less is more. Neuron 2013 0.77
70 If it's not one thing, it's another. Nat Genet 2006 0.76
71 Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study. Acta Neuropathol 2012 0.76
72 Dangerous liaisons: polyglutamine meets HMGB. Nat Cell Biol 2007 0.75
73 New hope for therapy in neurodegenerative diseases. Cell Res 2013 0.75
74 Drug discovery: Kill the messenger where it lives. Nature 2012 0.75
75 Considering a career in neurology research? Heed these words. Ann Neurol 2013 0.75