Rank |
Title |
Journal |
Year |
PubWeight™‹?› |
1
|
Parkinson's disease-associated mutations in leucine-rich repeat kinase 2 augment kinase activity.
|
Proc Natl Acad Sci U S A
|
2005
|
6.98
|
2
|
Kinase activity of mutant LRRK2 mediates neuronal toxicity.
|
Nat Neurosci
|
2006
|
5.32
|
3
|
Parkin mediates nonclassical, proteasomal-independent ubiquitination of synphilin-1: implications for Lewy body formation.
|
J Neurosci
|
2005
|
3.92
|
4
|
A schizophrenia-associated mutation of DISC1 perturbs cerebral cortex development.
|
Nat Cell Biol
|
2005
|
3.89
|
5
|
Preparing for preventive clinical trials: the Predict-HD study.
|
Arch Neurol
|
2006
|
3.57
|
6
|
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
|
7
|
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease.
|
Neuron
|
2005
|
3.13
|
8
|
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
|
9
|
Psychopathology in patients with degenerative cerebellar diseases: a comparison to Huntington's disease.
|
Am J Psychiatry
|
2002
|
2.52
|
10
|
Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease.
|
Brain
|
2007
|
2.17
|
11
|
Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization.
|
J Biol Chem
|
2002
|
2.12
|
12
|
A Drosophila model for LRRK2-linked parkinsonism.
|
Proc Natl Acad Sci U S A
|
2008
|
2.12
|
13
|
Prenatal interaction of mutant DISC1 and immune activation produces adult psychopathology.
|
Biol Psychiatry
|
2010
|
2.11
|
14
|
Acute kidney injury leads to inflammation and functional changes in the brain.
|
J Am Soc Nephrol
|
2008
|
2.03
|
15
|
Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets.
|
Nat Med
|
2011
|
1.97
|
16
|
Endoplasmic reticulum stress and mitochondrial cell death pathways mediate A53T mutant alpha-synuclein-induced toxicity.
|
Hum Mol Genet
|
2005
|
1.92
|
17
|
A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study.
|
Am J Hum Genet
|
2003
|
1.85
|
18
|
Striatal and white matter predictors of estimated diagnosis for Huntington disease.
|
Brain Res Bull
|
2010
|
1.79
|
19
|
"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
|
20
|
Alpha-synuclein phosphorylation enhances eosinophilic cytoplasmic inclusion formation in SH-SY5Y cells.
|
J Neurosci
|
2005
|
1.75
|
21
|
Motor abnormalities in premanifest persons with Huntington's disease: the PREDICT-HD study.
|
Mov Disord
|
2009
|
1.73
|
22
|
CHIP regulates leucine-rich repeat kinase-2 ubiquitination, degradation, and toxicity.
|
Proc Natl Acad Sci U S A
|
2009
|
1.64
|
23
|
Polyglutamine expansion of huntingtin impairs its nuclear export.
|
Nat Genet
|
2005
|
1.62
|
24
|
Huntington's disease--like 2 is associated with CUG repeat-containing RNA foci.
|
Ann Neurol
|
2007
|
1.61
|
25
|
Single particle characterization of iron-induced pore-forming alpha-synuclein oligomers.
|
J Biol Chem
|
2008
|
1.56
|
26
|
Verbal episodic memory declines prior to diagnosis in Huntington's disease.
|
Neuropsychologia
|
2007
|
1.54
|
27
|
Longitudinal change in regional brain volumes in prodromal Huntington disease.
|
J Neurol Neurosurg Psychiatry
|
2010
|
1.53
|
28
|
Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells.
|
J Biol Chem
|
2009
|
1.47
|
29
|
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
|
30
|
A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration.
|
J Neurosci
|
2004
|
1.45
|
31
|
Regional white matter change in pre-symptomatic Huntington's disease: a diffusion tensor imaging study.
|
Psychiatry Res
|
2005
|
1.43
|
32
|
Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length.
|
J Neurosci
|
2002
|
1.40
|
33
|
Astrocytes generated from patient induced pluripotent stem cells recapitulate features of Huntington's disease patient cells.
|
Mol Brain
|
2012
|
1.36
|
34
|
Smaller intracranial volume in prodromal Huntington's disease: evidence for abnormal neurodevelopment.
|
Brain
|
2010
|
1.35
|
35
|
Functional brain changes in presymptomatic Huntington's disease.
|
Ann Neurol
|
2004
|
1.34
|
36
|
Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects.
|
Hum Mol Genet
|
2002
|
1.33
|
37
|
N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease.
|
Cell Cycle
|
2007
|
1.31
|
38
|
Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study.
|
BMC Med Genet
|
2006
|
1.30
|
39
|
Ubiquitylation of synphilin-1 and alpha-synuclein by SIAH and its presence in cellular inclusions and Lewy bodies imply a role in Parkinson's disease.
|
Proc Natl Acad Sci U S A
|
2004
|
1.29
|
40
|
Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models.
|
J Neuropathol Exp Neurol
|
2007
|
1.25
|
41
|
A comparison of huntington disease and huntington disease-like 2 neuropathology.
|
J Neuropathol Exp Neurol
|
2008
|
1.24
|
42
|
The antidepressant sertraline improves the phenotype, promotes neurogenesis and increases BDNF levels in the R6/2 Huntington's disease mouse model.
|
Exp Neurol
|
2007
|
1.22
|
43
|
Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's disease.
|
Neurobiol Dis
|
2008
|
1.22
|
44
|
Cell death triggered by polyglutamine-expanded huntingtin in a neuronal cell line is associated with degradation of CREB-binding protein.
|
Hum Mol Genet
|
2003
|
1.22
|
45
|
Identification and functional characterization of a novel R621C mutation in the synphilin-1 gene in Parkinson's disease.
|
Hum Mol Genet
|
2003
|
1.22
|
46
|
Mutant huntingtin: nuclear translocation and cytotoxicity mediated by GAPDH.
|
Proc Natl Acad Sci U S A
|
2006
|
1.20
|
47
|
Synphilin-1 is developmentally localized to synaptic terminals, and its association with synaptic vesicles is modulated by alpha-synuclein.
|
J Biol Chem
|
2002
|
1.20
|
48
|
Disrupted-in-schizophrenia 1 and neuregulin 1 are required for the specification of oligodendrocytes and neurones in the zebrafish brain.
|
Hum Mol Genet
|
2008
|
1.19
|
49
|
Huntingtin phosphorylation sites mapped by mass spectrometry. Modulation of cleavage and toxicity.
|
J Biol Chem
|
2006
|
1.19
|
50
|
Evidence for a modifier of onset age in Huntington disease linked to the HD gene in 4p16.
|
Neurogenetics
|
2004
|
1.19
|
51
|
Cerebral cortex structure in prodromal Huntington disease.
|
Neurobiol Dis
|
2010
|
1.17
|
52
|
A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure.
|
Hum Mol Genet
|
2005
|
1.16
|
53
|
Longitudinal characterization of brain atrophy of a Huntington's disease mouse model by automated morphological analyses of magnetic resonance images.
|
Neuroimage
|
2009
|
1.15
|
54
|
The serum- and glucocorticoid-induced kinase SGK inhibits mutant huntingtin-induced toxicity by phosphorylating serine 421 of huntingtin.
|
Eur J Neurosci
|
2004
|
1.15
|
55
|
Depletion of CBP is directly linked with cellular toxicity caused by mutant huntingtin.
|
Neurobiol Dis
|
2006
|
1.14
|
56
|
A form of DISC1 enriched in nucleus: altered subcellular distribution in orbitofrontal cortex in psychosis and substance/alcohol abuse.
|
Proc Natl Acad Sci U S A
|
2005
|
1.14
|
57
|
Synphilin-1 attenuates neuronal degeneration in the A53T alpha-synuclein transgenic mouse model.
|
Hum Mol Genet
|
2010
|
1.12
|
58
|
Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice.
|
Hum Mol Genet
|
2004
|
1.11
|
59
|
Targeted disruption of Huntingtin-associated protein-1 (Hap1) results in postnatal death due to depressed feeding behavior.
|
Hum Mol Genet
|
2002
|
1.10
|
60
|
Striatal volume contributes to the prediction of onset of Huntington disease in incident cases.
|
Biol Psychiatry
|
2011
|
1.09
|
61
|
Huntingtin is cleaved by caspases in the cytoplasm and translocated to the nucleus via perinuclear sites in Huntington's disease patient lymphoblasts.
|
Neurobiol Dis
|
2005
|
1.08
|
62
|
Autophagosome-like vacuole formation in Huntington's disease lymphoblasts.
|
Neuroreport
|
2004
|
1.08
|
63
|
Small-molecule TrkB receptor agonists improve motor function and extend survival in a mouse model of Huntington's disease.
|
Hum Mol Genet
|
2013
|
1.08
|
64
|
Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein.
|
J Biol Chem
|
2012
|
1.07
|
65
|
Regionally selective atrophy of subcortical structures in prodromal HD as revealed by statistical shape analysis.
|
Hum Brain Mapp
|
2012
|
1.07
|
66
|
Structural MRI detects progressive regional brain atrophy and neuroprotective effects in N171-82Q Huntington's disease mouse model.
|
Neuroimage
|
2011
|
1.05
|
67
|
Common SNP-based haplotype analysis of the 4p16.3 Huntington disease gene region.
|
Am J Hum Genet
|
2012
|
1.05
|
68
|
Tiagabine is neuroprotective in the N171-82Q and R6/2 mouse models of Huntington's disease.
|
Neurobiol Dis
|
2008
|
1.05
|
69
|
Age, CAG repeat length, and clinical progression in Huntington's disease.
|
Mov Disord
|
2011
|
1.05
|
70
|
A compact beta model of huntingtin toxicity.
|
J Biol Chem
|
2011
|
1.04
|
71
|
Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease.
|
J Neurochem
|
2005
|
1.04
|
72
|
trans-(-)-ε-Viniferin increases mitochondrial sirtuin 3 (SIRT3), activates AMP-activated protein kinase (AMPK), and protects cells in models of Huntington Disease.
|
J Biol Chem
|
2012
|
1.02
|
73
|
FXTAS, SCA10, and SCA17 in American patients with movement disorders.
|
Am J Med Genet A
|
2005
|
1.01
|
74
|
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
|
75
|
Huntington's Disease-like 2 (HDL2) in North America and Japan.
|
Ann Neurol
|
2004
|
1.01
|
76
|
Dictyostelium huntingtin controls chemotaxis and cytokinesis through the regulation of myosin II phosphorylation.
|
Mol Biol Cell
|
2011
|
1.01
|
77
|
Polyglutamine repeat length-dependent proteolysis of huntingtin.
|
Neurobiol Dis
|
2002
|
1.00
|
78
|
Mutant huntingtin protein: a substrate for transglutaminase 1, 2, and 3.
|
J Neuropathol Exp Neurol
|
2005
|
1.00
|
79
|
Transcription meets metabolism in neurodegeneration.
|
Nat Med
|
2006
|
0.99
|
80
|
Compounds blocking mutant huntingtin toxicity identified using a Huntington's disease neuronal cell model.
|
Neurobiol Dis
|
2005
|
0.99
|
81
|
Environmental, pharmacological, and genetic modulation of the HD phenotype in transgenic mice.
|
Exp Neurol
|
2004
|
0.99
|
82
|
PC12 cell model of inducible expression of mutant DISC1: new evidence for a dominant-negative mechanism of abnormal neuronal differentiation.
|
Neurosci Res
|
2007
|
0.99
|
83
|
Parkinson's disease genetic mutations increase cell susceptibility to stress: mutant alpha-synuclein enhances H2O2- and Sin-1-induced cell death.
|
Neurobiol Aging
|
2006
|
0.99
|
84
|
Animal models of gene-environment interactions in schizophrenia.
|
Behav Brain Res
|
2009
|
0.98
|
85
|
Targeting H3K4 trimethylation in Huntington disease.
|
Proc Natl Acad Sci U S A
|
2013
|
0.98
|
86
|
Brain metabolite alterations and cognitive dysfunction in early Huntington's disease.
|
Mov Disord
|
2012
|
0.97
|
87
|
Inducible PC12 cell model of Huntington's disease shows toxicity and decreased histone acetylation.
|
Neuroreport
|
2003
|
0.97
|
88
|
Impaired cortico-striatal functional connectivity in prodromal Huntington's Disease.
|
Neurosci Lett
|
2012
|
0.97
|
89
|
Long tandem repeats as a form of genomic copy number variation: structure and length polymorphism of a chromosome 5p repeat in control and schizophrenia populations.
|
Psychiatr Genet
|
2009
|
0.96
|
90
|
Diagnosis of Huntington disease.
|
Clin Chem
|
2003
|
0.95
|
91
|
Neuroprotective effects of PPAR-γ agonist rosiglitazone in N171-82Q mouse model of Huntington's disease.
|
J Neurochem
|
2013
|
0.94
|
92
|
Prefrontal brain network connectivity indicates degree of both schizophrenia risk and cognitive dysfunction.
|
Schizophr Bull
|
2013
|
0.94
|
93
|
Primate disrupted-in-schizophrenia-1 (DISC1): high divergence of a gene for major mental illnesses in recent evolutionary history.
|
Neurosci Res
|
2006
|
0.94
|
94
|
Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity.
|
J Biol Chem
|
2002
|
0.93
|
95
|
Immunocytochemical localization of synphilin-1, an alpha-synuclein-associated protein, in neurodegenerative disorders.
|
Acta Neuropathol
|
2001
|
0.93
|
96
|
Neuropsychological deficits in Huntington's disease gene carriers and correlates of early "conversion".
|
J Neuropsychiatry Clin Neurosci
|
2008
|
0.92
|
97
|
Association between Age and Striatal Volume Stratified by CAG Repeat Length in Prodromal Huntington Disease.
|
PLoS Curr
|
2011
|
0.92
|
98
|
Transgenic mouse model expressing the caspase 6 fragment of mutant huntingtin.
|
J Neurosci
|
2012
|
0.92
|
99
|
Curcumin protects against A53T alpha-synuclein-induced toxicity in a PC12 inducible cell model for Parkinsonism.
|
Pharmacol Res
|
2011
|
0.91
|
100
|
Transglutaminase cross-links in intranuclear inclusions in Huntington disease.
|
J Neuropathol Exp Neurol
|
2003
|
0.91
|
101
|
A mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice.
|
Neurobiol Dis
|
2007
|
0.90
|
102
|
Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis.
|
Cell Cycle
|
2012
|
0.90
|
103
|
Seizures in juvenile Huntington's disease: frequency and characterization in a multicenter cohort.
|
Mov Disord
|
2012
|
0.90
|
104
|
Functional MRI study of a serial reaction time task in Huntington's disease.
|
Psychiatry Res
|
2004
|
0.90
|
105
|
Cysteine proteases bleomycin hydrolase and cathepsin Z mediate N-terminal proteolysis and toxicity of mutant huntingtin.
|
J Biol Chem
|
2011
|
0.90
|
106
|
Diagnostic criteria for Huntington's disease based on natural history.
|
Mov Disord
|
2014
|
0.90
|
107
|
Loss of junctophilin-3 contributes to Huntington disease-like 2 pathogenesis.
|
Ann Neurol
|
2012
|
0.89
|
108
|
Striatal neuronal loss correlates with clinical motor impairment in Huntington's disease.
|
Mov Disord
|
2012
|
0.89
|
109
|
Predictors of neuropathological severity in 100 patients with Huntington's disease.
|
Ann Neurol
|
2003
|
0.88
|
110
|
Factors contributing to institutionalization in patients with Huntington's disease.
|
Mov Disord
|
2011
|
0.87
|
111
|
Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice.
|
Hum Mol Genet
|
2008
|
0.86
|
112
|
Prefrontal executive function associated coupling relates to Huntington's disease stage.
|
Cortex
|
2013
|
0.84
|
113
|
Baicalein reduces E46K alpha-synuclein aggregation in vitro and protects cells against E46K alpha-synuclein toxicity in cell models of familiar Parkinsonism.
|
J Neurochem
|
2010
|
0.84
|
114
|
Population stratification may bias analysis of PGC-1α as a modifier of age at Huntington disease motor onset.
|
Hum Genet
|
2012
|
0.83
|
115
|
LRRK2 kinase activity mediates toxic interactions between genetic mutation and oxidative stress in a Drosophila model: suppression by curcumin.
|
Neurobiol Dis
|
2012
|
0.82
|
116
|
Calmodulin regulates transglutaminase 2 cross-linking of huntingtin.
|
J Neurosci
|
2004
|
0.81
|
117
|
Depressive symptoms in prodromal Huntington's Disease correlate with Stroop-interference related functional connectivity in the ventromedial prefrontal cortex.
|
Psychiatry Res
|
2012
|
0.81
|
118
|
TAA repeat variation in the GRIK2 gene does not influence age at onset in Huntington's disease.
|
Biochem Biophys Res Commun
|
2012
|
0.81
|
119
|
Gene expression alterations in bipolar disorder postmortem brains.
|
Bipolar Disord
|
2013
|
0.80
|
120
|
Potential therapeutic targets for neurodegenerative diseases: lessons learned from calorie restriction.
|
Curr Drug Targets
|
2010
|
0.80
|
121
|
Trinucleotide repeat expansions in the junctophilin-3 gene are not found in Caucasian patients with a Huntington's disease-like phenotype.
|
Ann Neurol
|
2002
|
0.80
|
122
|
PRMT5- mediated symmetric arginine dimethylation is attenuated by mutant huntingtin and is impaired in Huntington's disease (HD).
|
Cell Cycle
|
2015
|
0.80
|
123
|
Frontal cortical synaptic communication is abnormal in Disc1 genetic mouse models of schizophrenia.
|
Schizophr Res
|
2013
|
0.80
|
124
|
Cognitive impairment and psychiatric symptoms in 133 patients with diseases associated with cerebellar degeneration.
|
J Neuropsychiatry Clin Neurosci
|
2004
|
0.79
|
125
|
Spatiotemporal mapping of brain atrophy in mouse models of Huntington's disease using longitudinal in vivo magnetic resonance imaging.
|
Neuroimage
|
2012
|
0.79
|
126
|
Elevated arteriolar cerebral blood volume in prodromal Huntington's disease.
|
Mov Disord
|
2013
|
0.79
|
127
|
Intercellular miscommunication in polyglutamine pathogenesis.
|
Nat Neurosci
|
2006
|
0.78
|
128
|
ATF3 plays a protective role against toxicity by N-terminal fragment of mutant huntingtin in stable PC12 cell line.
|
Brain Res
|
2009
|
0.78
|
129
|
Bioactivity profiling with parallel mass spectrometry reveals an assemblage of green tea metabolites affording protection against human huntingtin and alpha-synuclein toxicity.
|
J Agric Food Chem
|
2007
|
0.78
|
130
|
Characterization of CTG/CAG repeats on chromosome 18: a study of bipolar disorder.
|
Psychiatr Genet
|
2005
|
0.78
|
131
|
Neuronal signaling pathways: genetic insights into the pathophysiology of major mental illness.
|
Neuropsychopharmacology
|
2010
|
0.77
|
132
|
Gross feature recognition of Anatomical Images based on Atlas grid (GAIA): Incorporating the local discrepancy between an atlas and a target image to capture the features of anatomic brain MRI.
|
Neuroimage Clin
|
2013
|
0.77
|
133
|
Candidate glutamatergic and dopaminergic pathway gene variants do not influence Huntington's disease motor onset.
|
Neurogenetics
|
2013
|
0.77
|
134
|
Diffuse abnormality of low to moderately organized white matter in schizophrenia.
|
Brain Connect
|
2011
|
0.77
|
135
|
Huntington's disease: a field on the move. Introduction.
|
Mov Disord
|
2014
|
0.76
|
136
|
Schizophrenia: A point of disruption.
|
Nature
|
2009
|
0.75
|
137
|
It is time to take a stand for medical research and against terrorism targeting medical scientists.
|
Biol Psychiatry
|
2008
|
0.75
|