Published in Amyotroph Lateral Scler on June 01, 2012
A novel small molecule HSP90 inhibitor, NXD30001, differentially induces heat shock proteins in nervous tissue in culture and in vivo. Cell Stress Chaperones (2013) 0.80
Exosome-dependent and independent mechanisms are involved in prion-like transmission of propagated Cu/Zn superoxide dismutase misfolding. Prion (2014) 0.78
Artifacts to avoid while taking advantage of top-down mass spectrometry based detection of protein S-thiolation. Proteomics (2014) 0.76
The Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded C9ORF72 Inhibits the Proteasome. eNeuro (2017) 0.75
Effects of Cellular Pathway Disturbances on Misfolded Superoxide Dismutase-1 in Fibroblasts Derived from ALS Patients. PLoS One (2016) 0.75
Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities? Front Mol Neurosci (2017) 0.75
Cellular Chaperones As Therapeutic Targets in ALS to Restore Protein Homeostasis and Improve Cellular Function. Front Mol Neurosci (2017) 0.75
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nat Neurosci (2010) 4.25
Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo. Hum Mol Genet (2009) 2.40
A soluble α-synuclein construct forms a dynamic tetramer. Proc Natl Acad Sci U S A (2011) 2.15
TMEM106B protects C9ORF72 expansion carriers against frontotemporal dementia. Acta Neuropathol (2014) 1.90
Mutations in COX10 result in a defect in mitochondrial heme A biosynthesis and account for multiple, early-onset clinical phenotypes associated with isolated COX deficiency. Hum Mol Genet (2003) 1.79
Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-up. Arch Neurol (2007) 1.78
Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury. Brain Res (2008) 1.77
High threshold for induction of the stress response in motor neurons is associated with failure to activate HSF1. J Neurosci (2003) 1.77
C9ORF72 repeat expansions in cases with previously identified pathogenic mutations. Neurology (2013) 1.70
Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival. PLoS Biol (2008) 1.61
Fitting neurological protein aggregation kinetic data via a 2-step, minimal/"Ockham's razor" model: the Finke-Watzky mechanism of nucleation followed by autocatalytic surface growth. Biochemistry (2008) 1.60
Ataxin-2 repeat-length variation and neurodegeneration. Hum Mol Genet (2011) 1.60
Molecular pathology and genetic advances in amyotrophic lateral sclerosis: an emerging molecular pathway and the significance of glial pathology. Acta Neuropathol (2011) 1.58
Local presynaptic activity gates homeostatic changes in presynaptic function driven by dendritic BDNF synthesis. Neuron (2010) 1.57
Progranulin is expressed within motor neurons and promotes neuronal cell survival. BMC Neurosci (2009) 1.46
Amyotrophic lateral sclerosis: update for family physicians. Can Fam Physician (2006) 1.45
NIPPV: prevalence, approach and barriers to use at Canadian ALS centres. Can J Neurol Sci (2010) 1.44
Clinical significance of imaging and histological characteristics of filum terminale in tethered cord syndrome. J Neurosurg Pediatr (2014) 1.44
Increasing peak expiratory flow time in amyotrophic lateral sclerosis. Chest (2005) 1.39
Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype. Neurobiol Aging (2012) 1.30
Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS. Neurosci Lett (2007) 1.28
Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations. Hum Mol Genet (2011) 1.27
Whole-genome sequencing of the Akata and Mutu Epstein-Barr virus strains. J Virol (2012) 1.26
Innate immunity in amyotrophic lateral sclerosis. Biochim Biophys Acta (2006) 1.26
Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis. J Neurochem (2004) 1.24
NifS-mediated assembly of [4Fe-4S] clusters in the N- and C-terminal domains of the NifU scaffold protein. Biochemistry (2005) 1.21
Optimized protocols for isolation of primary motor neurons, astrocytes and microglia from embryonic mouse spinal cord. J Neurosci Methods (2007) 1.19
Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis. Ann Neurol (2009) 1.17
Detection of murine leukemia virus in the Epstein-Barr virus-positive human B-cell line JY, using a computational RNA-Seq-based exogenous agent detection pipeline, PARSES. J Virol (2012) 1.14
Cytosolic TDP-43 expression following axotomy is associated with caspase 3 activation in NFL-/- mice: support for a role for TDP-43 in the physiological response to neuronal injury. Brain Res (2009) 1.14
Molecular imaging of drug transit through the blood-brain barrier with MALDI mass spectrometry imaging. Sci Rep (2013) 1.12
Prognosis of amyotrophic lateral sclerosis with respiratory onset. J Neurol Neurosurg Psychiatry (2006) 1.10
Characterization of detergent-insoluble proteins in ALS indicates a causal link between nitrative stress and aggregation in pathogenesis. PLoS One (2009) 1.10
Failure of protein quality control in amyotrophic lateral sclerosis. Biochim Biophys Acta (2006) 1.09
Sensitive and specific identification of wild type and variant proteins from 8 to 669 kDa using top-down mass spectrometry. Mol Cell Proteomics (2008) 1.09
A common property of amyotrophic lateral sclerosis-associated variants: destabilization of the copper/zinc superoxide dismutase electrostatic loop. J Biol Chem (2009) 1.08
Co-aggregation of RNA binding proteins in ALS spinal motor neurons: evidence of a common pathogenic mechanism. Acta Neuropathol (2012) 1.06
Induction of multiple heat shock proteins and neuroprotection in a primary culture model of familial amyotrophic lateral sclerosis. Neurobiol Dis (2006) 1.05
Matrix solution fixation: histology-compatible tissue preparation for MALDI mass spectrometry imaging. Anal Chem (2007) 1.05
Tryptophan 32 potentiates aggregation and cytotoxicity of a copper/zinc superoxide dismutase mutant associated with familial amyotrophic lateral sclerosis. J Biol Chem (2007) 1.05
Strategies for stabilizing superoxide dismutase (SOD1), the protein destabilized in the most common form of familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2010) 1.01
Activated microglia (BV-2) facilitation of TNF-alpha-mediated motor neuron death in vitro. J Neuroimmunol (2002) 1.00
Recent advances in single-cell MALDI mass spectrometry imaging and potential clinical impact. Expert Rev Proteomics (2011) 0.99
Post-transcriptional control of neurofilaments: New roles in development, regeneration and neurodegenerative disease. Trends Neurosci (2009) 0.98
Mitochondrial and axonal abnormalities precede disruption of the neurofilament network in a model of charcot-marie-tooth disease type 2E and are prevented by heat shock proteins in a mutant-specific fashion. J Neuropathol Exp Neurol (2009) 0.98
In vitro study of axonal migration and myelination of motor neurons in a three-dimensional tissue-engineered model. Glia (2008) 0.98
An aggregate-inducing peripherin isoform generated through intron retention is upregulated in amyotrophic lateral sclerosis and associated with disease pathology. J Neurosci (2008) 0.97
Mutant copper-zinc superoxide dismutase binds to and destabilizes human low molecular weight neurofilament mRNA. J Biol Chem (2004) 0.96
Activated p38MAPK is a novel component of the intracellular inclusions found in human amyotrophic lateral sclerosis and mutant SOD1 transgenic mice. J Neuropathol Exp Neurol (2004) 0.96
Altered microRNA expression profile in Amyotrophic Lateral Sclerosis: a role in the regulation of NFL mRNA levels. Mol Brain (2013) 0.95
A neurotoxic peripherin splice variant in a mouse model of ALS. J Cell Biol (2003) 0.95
A hierarchical algorithm for calculating the isotopic fine structures of molecules. J Am Soc Mass Spectrom (2008) 0.95
Quantitative phosphoproteomic analysis of neuronal intermediate filament proteins (NF-M/H) in Alzheimer's disease by iTRAQ. FASEB J (2010) 0.95
Structural characterization of intact proteins is enhanced by prevalent fragmentation pathways rarely observed for peptides. J Am Soc Mass Spectrom (2010) 0.94
Inhibition of Pin1 reduces glutamate-induced perikaryal accumulation of phosphorylated neurofilament-H in neurons. Mol Biol Cell (2007) 0.94
Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: mechanistic relationship and differential sensitivity to intervention. Neurobiol Dis (2011) 0.93
Global bidirectional transcription of the Epstein-Barr virus genome during reactivation. J Virol (2013) 0.93
Proteasomes remain intact, but show early focal alteration in their composition in a mouse model of amyotrophic lateral sclerosis. J Neurochem (2008) 0.92
Imaging of meningioma progression by matrix-assisted laser desorption ionization time-of-flight mass spectrometry. Anal Chem (2010) 0.91
Detection of a novel frameshift mutation and regions with homozygosis within ARHGEF28 gene in familial amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener (2013) 0.90
Effects of the endocrine-disrupting chemical DDT on self-renewal and differentiation of human mesenchymal stem cells. Environ Health Perspect (2014) 0.90
Normal role of the low-molecular-weight neurofilament protein in mitochondrial dynamics and disruption in Charcot-Marie-Tooth disease. FASEB J (2011) 0.90
Microtubule-associated tau protein positive neuronal and glial inclusions in ALS. Neurology (2003) 0.89
Post-transcriptional control of neurofilaments in development and disease. Exp Cell Res (2007) 0.88
Evidence of the participation of remote residues in the catalytic activity of Co-type nitrile hydratase from Pseudomonas putida. Biochemistry (2011) 0.87
In vitro development of a tissue-engineered model of peripheral nerve regeneration to study neurite growth. FASEB J (2003) 0.87
Widespread neuronal and glial hyperphosphorylated tau deposition in ALS with cognitive impairment. Amyotroph Lateral Scler (2012) 0.87
NMDA induces NOS 1 translocation to the cell membrane in NGF-differentiated PC 12 cells. Brain Res (2003) 0.87
Post-translational modification by cysteine protects Cu/Zn-superoxide dismutase from oxidative damage. Biochemistry (2013) 0.86
Calpastatin reduces toxicity of SOD1G93A in a culture model of amyotrophic lateral sclerosis. Neuroreport (2010) 0.86
Neuropsychological functioning in PLS: a comparison with ALS. Can J Neurol Sci (2011) 0.85
Transient middle cerebral artery occlusion induces microglial priming in the lumbar spinal cord: a novel model of neuroinflammation. J Neuroinflammation (2008) 0.85
Calcium mediated excitotoxicity in neurofilament aggregate-bearing neurons in vitro is NMDA receptor dependant. J Neurol Sci (2007) 0.85
The complement factor C5a receptor is upregulated in NFL-/- mouse motor neurons. J Neuroimmunol (2009) 0.84
Genetic modifiers in carriers of repeat expansions in the C9ORF72 gene. Mol Neurodegener (2014) 0.84
Selective loss of trans-acting instability determinants of neurofilament mRNA in amyotrophic lateral sclerosis spinal cord. J Biol Chem (2003) 0.84
Tau phosphorylation at threonine-175 leads to fibril formation and enhanced cell death: implications for amyotrophic lateral sclerosis with cognitive impairment. J Neurochem (2008) 0.83
Activated microglial supernatant induced motor neuron cytotoxicity is associated with upregulation of the TNFR1 receptor. Neurosci Res (2006) 0.83
Upregulation of GSK3beta expression in frontal and temporal cortex in ALS with cognitive impairment (ALSci). Brain Res (2008) 0.83
Characterizing the role of Hsp90 in production of heat shock proteins in motor neurons reveals a suppressive effect of wild-type Hsf1. Cell Stress Chaperones (2007) 0.83
Temporal profiles of neuronal degeneration, glial proliferation, and cell death in hNFL(+/+) and NFL(-/-) mice. Glia (2005) 0.82
14-3-3 protein binds to the low molecular weight neurofilament (NFL) mRNA 3' UTR. Mol Cell Neurosci (2006) 0.82
Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease. Neurotherapeutics (2015) 0.81
RNA metabolism in ALS: when normal processes become pathological. Amyotroph Lateral Scler Frontotemporal Degener (2014) 0.81
Manipulation of protein kinases reveals different mechanisms for upregulation of heat shock proteins in motor neurons and non-neuronal cells. Mol Cell Neurosci (2006) 0.81
A novel small molecule HSP90 inhibitor, NXD30001, differentially induces heat shock proteins in nervous tissue in culture and in vivo. Cell Stress Chaperones (2013) 0.80
Genetic analysis of SIGMAR1 as a cause of familial ALS with dementia. Eur J Hum Genet (2012) 0.80
Human low molecular weight neurofilament (NFL) mRNA interacts with a predicted p190RhoGEF homologue (RGNEF) in humans. Amyotroph Lateral Scler (2010) 0.80
Structural consequences of cysteinylation of Cu/Zn-superoxide dismutase. Biochemistry (2013) 0.80
Tau protein aggregation in the frontal and entorhinal cortices as a function of aging. Brain Res Dev Brain Res (2005) 0.80
Cerebral haemodynamic changes accompanying cognitive impairment in primary lateral sclerosis. Amyotroph Lateral Scler (2008) 0.80
Creutzfeldt-Jakob disease presenting with visual manifestations. Can J Ophthalmol (2008) 0.79
Neurofilament dynamics and involvement in neurological disorders. Cell Tissue Res (2015) 0.79
Rho guanine nucleotide exchange factor is an NFL mRNA destabilizing factor that forms cytoplasmic inclusions in amyotrophic lateral sclerosis. Neurobiol Aging (2012) 0.79