1
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Regulation of insulin biosynthesis in pancreatic beta cells by an endoplasmic reticulum-resident protein kinase IRE1.
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Cell Metab
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2006
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4.11
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2
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Thioredoxin-interacting protein mediates ER stress-induced β cell death through initiation of the inflammasome.
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Cell Metab
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2012
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2.39
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3
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Wolfram syndrome 1 gene negatively regulates ER stress signaling in rodent and human cells.
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J Clin Invest
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2010
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2.22
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4
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Differential expression of inflammation- and apoptosis-related genes in spinal cords of a mutant SOD1 transgenic mouse model of familial amyotrophic lateral sclerosis.
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J Neurochem
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2002
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1.63
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5
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Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis.
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Ann Neurol
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2005
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1.59
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6
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Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1.
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J Biol Chem
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2007
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1.57
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7
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Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity.
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J Biol Chem
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2002
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1.19
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8
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Loss of TDP-43 causes age-dependent progressive motor neuron degeneration.
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Brain
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2013
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1.05
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9
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Mitochondrial localization of mutant superoxide dismutase 1 triggers caspase-dependent cell death in a cellular model of familial amyotrophic lateral sclerosis.
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J Biol Chem
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2002
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1.03
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10
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Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies.
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Neurobiol Dis
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2011
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0.99
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11
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FUS/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress.
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J Cell Physiol
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2013
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0.96
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12
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Dorfin localizes to Lewy bodies and ubiquitylates synphilin-1.
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J Biol Chem
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2003
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0.95
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13
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Naratriptan mitigates CGRP1-associated motor neuron degeneration caused by an expanded polyglutamine repeat tract.
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Nat Med
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2012
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0.91
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14
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Disruption of axonal transport in motor neuron diseases.
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Int J Mol Sci
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2012
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0.89
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15
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Amyotrophic lateral sclerosis: an update on recent genetic insights.
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J Neurol
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2013
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0.89
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16
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Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration.
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Nat Commun
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2013
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0.88
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17
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Valproate, a mood stabilizer, induces WFS1 expression and modulates its interaction with ER stress protein GRP94.
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PLoS One
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2009
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0.88
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18
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Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis.
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J Neurochem
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2004
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0.86
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19
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FUS-regulated region- and cell-type-specific transcriptome is associated with cell selectivity in ALS/FTLD.
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Sci Rep
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2013
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0.85
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20
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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.
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J Neuropathol Exp Neurol
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2007
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0.84
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21
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The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons.
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FEBS Open Bio
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2013
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0.83
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22
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Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis.
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J Neurosci Res
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2010
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0.82
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23
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Dorfin localizes to the ubiquitylated inclusions in Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and amyotrophic lateral sclerosis.
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Am J Pathol
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2003
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0.81
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24
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Gene expression profiling toward understanding of ALS pathogenesis.
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Ann N Y Acad Sci
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2006
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0.81
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25
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The principal PINK1 and Parkin cellular events triggered in response to dissipation of mitochondrial membrane potential occur in primary neurons.
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Genes Cells
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2013
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0.80
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26
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Establishment of a system for monitoring endoplasmic reticulum redox state in mammalian cells.
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Lab Invest
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2013
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0.79
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27
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c-Abl inhibition delays motor neuron degeneration in the G93A mouse, an animal model of amyotrophic lateral sclerosis.
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PLoS One
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2012
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0.79
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28
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RNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43.
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PLoS One
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2013
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0.79
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29
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Caspase-1 and -3 mRNAs are differentially upregulated in motor neurons and glial cells in mutant SOD1 transgenic mouse spinal cord: a study using laser microdissection and real-time RT-PCR.
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Neurochem Res
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2003
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0.77
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30
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Archaeal proteasomes effectively degrade aggregation-prone proteins and reduce cellular toxicities in mammalian cells.
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J Biol Chem
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2006
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0.75
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31
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Mitochondrial localization of mutant superoxide dismutase 1 triggers caspase-dependent cell death in a cellular model of familial amyotrophic lateral sclerosis.
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J Biol Chem
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2016
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0.75
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32
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Gene Expression Profile of Inflammatory Myopathy with Malignancy is Similar to that of Dermatomyositis rather than Polymyositis.
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Intern Med
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2016
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0.75
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33
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[Motor neuron disease up-to date].
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No To Shinkei
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2005
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0.75
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34
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Transcriptional and post-transcriptional regulation of transmembrane protein 132A.
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Mol Cell Biochem
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2015
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0.75
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