| Rank |
Title |
Journal |
Year |
PubWeight™‹?› |
|
1
|
The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy.
|
Brain
|
2005
|
3.65
|
|
2
|
17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration.
|
Nat Med
|
2005
|
3.19
|
|
3
|
Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy.
|
Neuron
|
2009
|
2.26
|
|
4
|
Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis.
|
Ann Neurol
|
2005
|
1.59
|
|
5
|
Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions.
|
Sci Rep
|
2012
|
1.58
|
|
6
|
Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patients.
|
Brain
|
2006
|
1.57
|
|
7
|
Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1.
|
J Biol Chem
|
2007
|
1.57
|
|
8
|
Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein.
|
J Neurosci
|
2003
|
1.54
|
|
9
|
Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy.
|
Hum Mol Genet
|
2004
|
1.52
|
|
10
|
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases.
|
J Biol Chem
|
2009
|
1.44
|
|
11
|
Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy.
|
Ann Neurol
|
2009
|
1.43
|
|
12
|
Viral delivery of miR-196a ameliorates the SBMA phenotype via the silencing of CELF2.
|
Nat Med
|
2012
|
1.41
|
|
13
|
CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model.
|
J Neurosci
|
2007
|
1.34
|
|
14
|
Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients.
|
Brain
|
2005
|
1.33
|
|
15
|
17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse.
|
Hum Mol Genet
|
2008
|
1.25
|
|
16
|
Modulation of Hsp90 function in neurodegenerative disorders: a molecular-targeted therapy against disease-causing protein.
|
J Mol Med (Berl)
|
2006
|
1.16
|
|
17
|
Suppression of Alzheimer's disease-related phenotypes by expression of heat shock protein 70 in mice.
|
J Neurosci
|
2011
|
1.14
|
|
18
|
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration.
|
Brain
|
2013
|
1.05
|
|
19
|
CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA.
|
Brain
|
2007
|
1.04
|
|
20
|
Correlation between pyramidal tract degeneration and widespread white matter involvement in amyotrophic lateral sclerosis: a study with tractography and diffusion-tensor imaging.
|
Amyotroph Lateral Scler
|
2009
|
1.03
|
|
21
|
Macroautophagy is regulated by the UPR-mediator CHOP and accentuates the phenotype of SBMA mice.
|
PLoS Genet
|
2011
|
1.02
|
|
22
|
Microarray analysis of gene expression by skeletal muscle of three mouse models of Kennedy disease/spinal bulbar muscular atrophy.
|
PLoS One
|
2010
|
1.02
|
|
23
|
Clinicopathological features of acute autonomic and sensory neuropathy.
|
Brain
|
2010
|
1.00
|
|
24
|
Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies.
|
Neurobiol Dis
|
2011
|
0.99
|
|
25
|
Walking capacity evaluated by the 6-minute walk test in spinal and bulbar muscular atrophy.
|
Muscle Nerve
|
2008
|
0.97
|
|
26
|
Alleviating neurodegeneration by an anticancer agent: an Hsp90 inhibitor (17-AAG).
|
Ann N Y Acad Sci
|
2006
|
0.96
|
|
27
|
Insulinlike growth factor (IGF)-1 administration ameliorates disease manifestations in a mouse model of spinal and bulbar muscular atrophy.
|
Mol Med
|
2012
|
0.96
|
|
28
|
p62/SQSTM1 differentially removes the toxic mutant androgen receptor via autophagy and inclusion formation in a spinal and bulbar muscular atrophy mouse model.
|
J Neurosci
|
2013
|
0.95
|
|
29
|
Transcriptional activation of TFEB/ZKSCAN3 target genes underlies enhanced autophagy in spinobulbar muscular atrophy.
|
Hum Mol Genet
|
2013
|
0.94
|
|
30
|
dnc-1/dynactin 1 knockdown disrupts transport of autophagosomes and induces motor neuron degeneration.
|
PLoS One
|
2013
|
0.94
|
|
31
|
Geranylgeranylacetone ameliorates ischemic acute renal failure via induction of Hsp70.
|
Kidney Int
|
2005
|
0.93
|
|
32
|
Intravenous immunoglobulin treatment for painful sensory neuropathy associated with Sjögren's syndrome.
|
J Neurol Sci
|
2009
|
0.92
|
|
33
|
Neuronal intranuclear inclusion disease cases with leukoencephalopathy diagnosed via skin biopsy.
|
J Neurol Neurosurg Psychiatry
|
2013
|
0.92
|
|
34
|
Naratriptan mitigates CGRP1-associated motor neuron degeneration caused by an expanded polyglutamine repeat tract.
|
Nat Med
|
2012
|
0.91
|
|
35
|
Distinct characteristics of amyloid deposits in early- and late-onset transthyretin Val30Met familial amyloid polyneuropathy.
|
J Neurol Sci
|
2009
|
0.89
|
|
36
|
Disruption of axonal transport in motor neuron diseases.
|
Int J Mol Sci
|
2012
|
0.89
|
|
37
|
Amyotrophic lateral sclerosis: an update on recent genetic insights.
|
J Neurol
|
2013
|
0.89
|
|
38
|
Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration.
|
Nat Commun
|
2013
|
0.88
|
|
39
|
A functional variant in ZNF512B is associated with susceptibility to amyotrophic lateral sclerosis in Japanese.
|
Hum Mol Genet
|
2011
|
0.86
|
|
40
|
Tongue pressure as a novel biomarker of spinal and bulbar muscular atrophy.
|
Neurology
|
2013
|
0.85
|
|
41
|
FUS-regulated region- and cell-type-specific transcriptome is associated with cell selectivity in ALS/FTLD.
|
Sci Rep
|
2013
|
0.85
|
|
42
|
Cortical and subcortical brain atrophy in Parkinson's disease with visual hallucination.
|
Mov Disord
|
2013
|
0.85
|
|
43
|
Longitudinal changes of outcome measures in spinal and bulbar muscular atrophy.
|
Brain
|
2012
|
0.84
|
|
44
|
TSUNAMI: an antisense method to phenocopy splicing-associated diseases in animals.
|
Genes Dev
|
2012
|
0.84
|
|
45
|
Gene expressions specifically detected in motor neurons (dynactin 1, early growth response 3, acetyl-CoA transporter, death receptor 5, and cyclin C) differentially correlate to pathologic markers in sporadic amyotrophic lateral sclerosis.
|
J Neuropathol Exp Neurol
|
2007
|
0.84
|
|
46
|
Mutant androgen receptor accumulation in spinal and bulbar muscular atrophy scrotal skin: a pathogenic marker.
|
Ann Neurol
|
2006
|
0.83
|
|
47
|
Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients.
|
J Neurol Neurosurg Psychiatry
|
2013
|
0.83
|
|
48
|
The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons.
|
FEBS Open Bio
|
2013
|
0.83
|
|
49
|
Heat shock proteins in neurodegenerative diseases: pathogenic roles and therapeutic implications.
|
Int J Hyperthermia
|
2009
|
0.83
|
|
50
|
Spatial distribution of nerve fiber pathology and vasculitis in microscopic polyangiitis-associated neuropathy.
|
J Neuropathol Exp Neurol
|
2011
|
0.83
|
|
51
|
Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis.
|
J Neurosci Res
|
2010
|
0.82
|
|
52
|
Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients.
|
Amyotroph Lateral Scler Frontotemporal Degener
|
2014
|
0.81
|
|
53
|
Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy (SBMA).
|
Cell Tissue Res
|
2012
|
0.81
|
|
54
|
Demographic features of Japanese patients with sporadic inclusion body myositis: a single-center referral experience.
|
Intern Med
|
2013
|
0.81
|
|
55
|
Gene expression profiling toward understanding of ALS pathogenesis.
|
Ann N Y Acad Sci
|
2006
|
0.81
|
|
56
|
Genistein, a natural product derived from soybeans, ameliorates polyglutamine-mediated motor neuron disease.
|
J Neurochem
|
2013
|
0.81
|
|
57
|
Antiandrogen flutamide protects male mice from androgen-dependent toxicity in three models of spinal bulbar muscular atrophy.
|
Endocrinology
|
2014
|
0.81
|
|
58
|
Differential, size-dependent sensory neuron involvement in the painful and ataxic forms of primary Sjögren's syndrome-associated neuropathy.
|
J Neurol Sci
|
2012
|
0.80
|
|
59
|
Discrimination of spinal and bulbar muscular atrophy from amyotrophic lateral sclerosis using sensory nerve action potentials.
|
Muscle Nerve
|
2012
|
0.80
|
|
60
|
Putaminal magnetic resonance imaging features at various magnetic field strengths in multiple system atrophy.
|
Mov Disord
|
2010
|
0.79
|
|
61
|
RNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43.
|
PLoS One
|
2013
|
0.79
|
|
62
|
c-Abl inhibition delays motor neuron degeneration in the G93A mouse, an animal model of amyotrophic lateral sclerosis.
|
PLoS One
|
2012
|
0.79
|
|
63
|
Neuropathology and therapeutic intervention in spinal and bulbar muscular atrophy.
|
Int J Mol Sci
|
2009
|
0.78
|
|
64
|
Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A.
|
J Neuropathol Exp Neurol
|
2007
|
0.78
|
|
65
|
Systemic but asymptomatic transthyretin amyloidosis 8 years after domino liver transplantation.
|
J Neurol Neurosurg Psychiatry
|
2010
|
0.78
|
|
66
|
IgM MGUS anti-MAG neuropathy with predominant muscle weakness and extensive muscle atrophy.
|
Muscle Nerve
|
2010
|
0.78
|
|
67
|
Sweet relief for Huntington disease.
|
Nat Med
|
2004
|
0.78
|
|
68
|
Current status of treatment of spinal and bulbar muscular atrophy.
|
Neural Plast
|
2012
|
0.78
|
|
69
|
Severe brain atrophy in the elderly as a risk factor for lower respiratory tract infection.
|
Clin Interv Aging
|
2012
|
0.77
|
|
70
|
Morphological progression of myelin abnormalities in IgM-monoclonal gammopathy of undetermined significance anti-myelin-associated glycoprotein neuropathy.
|
J Neuropathol Exp Neurol
|
2010
|
0.77
|
|
71
|
Difference in chronological changes of outcome measures between untreated and placebo-treated patients of spinal and bulbar muscular atrophy.
|
J Neurol
|
2011
|
0.77
|
|
72
|
[Molecular-targeted therapy for motor neuron disease].
|
Brain Nerve
|
2009
|
0.77
|
|
73
|
Head Lift Exercise Improves Swallowing Dysfunction in Spinal and Bulbar Muscular Atrophy.
|
Eur Neurol
|
2015
|
0.77
|
|
74
|
Slowly progressive autonomic neuropathy with antiganglionic acetylcholine receptor antibody.
|
J Neurol Neurosurg Psychiatry
|
2010
|
0.76
|
|
75
|
Dutasteride for spinal and bulbar muscular atrophy.
|
Lancet Neurol
|
2011
|
0.76
|
|
76
|
The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy.
|
J Neurol Neurosurg Psychiatry
|
2009
|
0.76
|
|
77
|
Intravenous immunoglobulin therapy in proximal diabetic neuropathy.
|
BMJ Case Rep
|
2009
|
0.75
|
|
78
|
Alcoholic Myelopathy and Nutritional Deficiency.
|
Intern Med
|
2017
|
0.75
|
|
79
|
Impaired Pain Processing Correlates with Cognitive Impairment in Parkinson's Disease.
|
Intern Med
|
2016
|
0.75
|
|
80
|
Progressive multifocal leukoencephalopathy with bilateral middle cerebellar peduncle lesions confirmed by repeated CSF-JC virus tests and coexistence of JC virus granule cell neuronopathy. Report of a case.
|
Rinsho Shinkeigaku
|
2016
|
0.75
|
|
81
|
[67Ga scintigraphy as a therapeutic marker for spinal cord and muscular sarcoidosis: A case report].
|
Rinsho Shinkeigaku
|
2015
|
0.75
|
|
82
|
Expanding concept of clinical conditions and symptoms in multiple system atrophy.
|
Rinsho Shinkeigaku
|
2016
|
0.75
|
|
83
|
Pathological background of subcortical hyperintensities on diffusion-weighted images in a case of neuronal intranuclear inclusion disease.
|
Clin Neuropathol
|
2016
|
0.75
|
|
84
|
Gene Expression Profile of Inflammatory Myopathy with Malignancy is Similar to that of Dermatomyositis rather than Polymyositis.
|
Intern Med
|
2016
|
0.75
|
|
85
|
[Development of the treatment for bulbospinal muscular atrophy].
|
Nihon Naika Gakkai Zasshi
|
2004
|
0.75
|
|
86
|
Cross-sectional and longitudinal analysis of an oxidative stress biomarker for spinal and bulbar muscular atrophy.
|
Muscle Nerve
|
2012
|
0.75
|
|
87
|
Schwann cell involvement in the peripheral neuropathy of spinocerebellar ataxia type 3.
|
Neuropathol Appl Neurobiol
|
2014
|
0.75
|
|
88
|
Polymorphism of transient axonal glycoprotein-1 in chronic inflammatory demyelinating polyneuropathy.
|
J Peripher Nerv Syst
|
2011
|
0.75
|
|
89
|
[Disease-modifying therapy for spinal and bulbar muscular atrophy (SBMA)].
|
Brain Nerve
|
2012
|
0.75
|
|
90
|
[Treatment of spinal and bulbar muscular atrophy].
|
No To Shinkei
|
2004
|
0.75
|
|
91
|
Acute superficial sensory neuropathy with generalized anhidrosis, anosmia, and ageusia.
|
Muscle Nerve
|
2011
|
0.75
|
|
92
|
[Motor neuron disease up-to date].
|
No To Shinkei
|
2005
|
0.75
|