Published in Brain on May 04, 2005
Stress-activated cap'n'collar transcription factors in aging and human disease. Sci Signal (2010) 3.19
Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis. Nat Rev Neurol (2011) 2.73
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1. Genes Dev (2008) 2.43
The biology of proteostasis in aging and disease. Annu Rev Biochem (2015) 1.96
Gene expression profiling in human neurodegenerative disease. Nat Rev Neurol (2012) 1.59
Thioredoxins, glutaredoxins, and peroxiredoxins--molecular mechanisms and health significance: from cofactors to antioxidants to redox signaling. Antioxid Redox Signal (2013) 1.48
Progranulin is expressed within motor neurons and promotes neuronal cell survival. BMC Neurosci (2009) 1.46
Emerging roles of Nrf2 and phase II antioxidant enzymes in neuroprotection. Prog Neurobiol (2012) 1.41
Beneficial role of Nrf2 in regulating NADPH generation and consumption. Toxicol Sci (2011) 1.41
Granulocyte-colony stimulating factor improves outcome in a mouse model of amyotrophic lateral sclerosis. Brain (2008) 1.40
Nrf2 impacts cellular bioenergetics by controlling substrate availability for mitochondrial respiration. Biol Open (2013) 1.20
Mechanisms, models and biomarkers in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener (2013) 1.16
MTOR-independent, autophagic enhancer trehalose prolongs motor neuron survival and ameliorates the autophagic flux defect in a mouse model of amyotrophic lateral sclerosis. Autophagy (2014) 1.09
Nrf2/ARE Signaling Pathway: Key Mediator in Oxidative Stress and Potential Therapeutic Target in ALS. Neurol Res Int (2012) 1.02
Phosphatase and tensin homologue/protein kinase B pathway linked to motor neuron survival in human superoxide dismutase 1-related amyotrophic lateral sclerosis. Brain (2011) 1.00
NRF2-regulation in brain health and disease: implication of cerebral inflammation. Neuropharmacology (2013) 1.00
Dysregulation of glucose metabolism is an early event in sporadic Parkinson's disease. Neurobiol Aging (2013) 0.99
Can transcriptomics cut the gordian knot of amyotrophic lateral sclerosis? Curr Genomics (2011) 0.94
SOD1 and DJ-1 converge at Nrf2 pathway: a clue for antioxidant therapeutic potential in neurodegeneration. Oxid Med Cell Longev (2013) 0.93
Cellular interplay between neurons and glia: toward a comprehensive mechanism for excitotoxic neuronal loss in neurodegeneration. Cellscience (2007) 0.92
SMN protects cells against mutant SOD1 toxicity by increasing chaperone activity. Biochem Biophys Res Commun (2007) 0.90
State of the field: An informatics-based systematic review of the SOD1-G93A amyotrophic lateral sclerosis transgenic mouse model. Amyotroph Lateral Scler Frontotemporal Degener (2015) 0.89
Induction of phase 2 antioxidant enzymes by broccoli sulforaphane: perspectives in maintaining the antioxidant activity of vitamins a, C, and e. Front Genet (2012) 0.87
Histone deacetylases and their role in motor neuron degeneration. Front Cell Neurosci (2013) 0.86
Redox proteomics in some age-related neurodegenerative disorders or models thereof. NeuroRx (2006) 0.86
An in vitro screening cascade to identify neuroprotective antioxidants in ALS. Free Radic Biol Med (2009) 0.86
C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis. PLoS One (2015) 0.86
Viral delivery of antioxidant genes as a therapeutic strategy in experimental models of amyotrophic lateral sclerosis. Mol Ther (2013) 0.85
Target- and mechanism-based therapeutics for neurodegenerative diseases: strength in numbers. J Med Chem (2013) 0.84
Changes in the spinal cord proteome of an amyotrophic lateral sclerosis murine model determined by differential in-gel electrophoresis. Mol Cell Proteomics (2009) 0.84
Gene expression signatures in motor neurone disease fibroblasts reveal dysregulation of metabolism, hypoxia-response and RNA processing functions. Neuropathol Appl Neurobiol (2015) 0.84
Human axonal survival of motor neuron (a-SMN) protein stimulates axon growth, cell motility, C-C motif ligand 2 (CCL2), and insulin-like growth factor-1 (IGF1) production. J Biol Chem (2012) 0.84
S[+] Apomorphine is a CNS penetrating activator of the Nrf2-ARE pathway with activity in mouse and patient fibroblast models of amyotrophic lateral sclerosis. Free Radic Biol Med (2013) 0.83
TorsinA rescues ER-associated stress and locomotive defects in C. elegans models of ALS. Dis Model Mech (2013) 0.82
Investigating cell death mechanisms in amyotrophic lateral sclerosis using transcriptomics. Front Cell Neurosci (2013) 0.82
Diverse expression of selected SMN complex proteins in humans with sporadic amyotrophic lateral sclerosis and in a transgenic rat model of familial form of the disease. PLoS One (2014) 0.79
The effect of SOD1 mutation on cellular bioenergetic profile and viability in response to oxidative stress and influence of mutation-type. PLoS One (2013) 0.79
Screening of the transcriptional regulatory regions of vascular endothelial growth factor receptor 2 (VEGFR2) in amyotrophic lateral sclerosis. BMC Med Genet (2007) 0.78
Cell Death-Autophagy Loop and Glutamate-Glutamine Cycle in Amyotrophic Lateral Sclerosis. Front Mol Neurosci (2017) 0.77
From Transcriptome to Noncoding RNAs: Implications in ALS Mechanism. Neurol Res Int (2012) 0.76
Transcriptome Profiling Following Neuronal and Glial Expression of ALS-Linked SOD1 in Drosophila. G3 (Bethesda) (2013) 0.76
A screen to identify cellular modulators of soluble levels of an amyotrophic lateral sclerosis (ALS)-causing mutant SOD1. J Biomol Screen (2011) 0.76
Motor neurons derived from ALS-related mouse iPS cells recapitulate pathological features of ALS. Exp Mol Med (2016) 0.75
Amyotrophic lateral sclerosis, gene deregulation in the anterior horn of the spinal cord and frontal cortex area 8: implications in frontotemporal lobar degeneration. Aging (Albany NY) (2017) 0.75
Report on the 3rd Ottawa International Conference on Neuromuscular Biology, Disease and Therapy - September 24-26, 2015, Ottawa, Canada. J Neuromuscul Dis (2016) 0.75
ALS Pathogenesis and Therapeutic Approaches: The Role of Mesenchymal Stem Cells and Extracellular Vesicles. Front Cell Neurosci (2017) 0.75
MicroNeurotrophins Improve Survival in Motor Neuron-Astrocyte Co-Cultures but Do Not Improve Disease Phenotypes in a Mutant SOD1 Mouse Model of Amyotrophic Lateral Sclerosis. PLoS One (2016) 0.75
Plant toxin β-ODAP activates integrin β1 and focal adhesion: A critical pathway to cause neurolathyrism. Sci Rep (2017) 0.75
Are Astrocytes the Predominant Cell Type for Activation of Nrf2 in Aging and Neurodegeneration? Antioxidants (Basel) (2017) 0.75
Multi-site assessment of the precision and reproducibility of multiple reaction monitoring-based measurements of proteins in plasma. Nat Biotechnol (2009) 9.67
Oxidative DNA damage: mechanisms, mutation, and disease. FASEB J (2003) 7.21
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. Ann Neurol (2007) 6.58
Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. Nat Genet (2010) 5.52
The Parkinson's disease protein DJ-1 is neuroprotective due to cysteine-sulfinic acid-driven mitochondrial localization. Proc Natl Acad Sci U S A (2004) 4.76
Adaptation to culture of human embryonic stem cells and oncogenesis in vivo. Nat Biotechnol (2007) 4.53
Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol (2006) 4.17
A transgenic zebrafish model of neutrophilic inflammation. Blood (2006) 3.93
Chromosome 9p21 in sporadic amyotrophic lateral sclerosis in the UK and seven other countries: a genome-wide association study. Lancet Neurol (2010) 3.61
White matter lesions in an unselected cohort of the elderly: molecular pathology suggests origin from chronic hypoperfusion injury. Stroke (2006) 3.27
Chromosome 9 ALS and FTD locus is probably derived from a single founder. Neurobiol Aging (2011) 3.27
Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis. Nat Rev Neurol (2011) 2.73
Controversies and priorities in amyotrophic lateral sclerosis. Lancet Neurol (2013) 2.52
Safety and efficacy of diaphragm pacing in patients with respiratory insufficiency due to amyotrophic lateral sclerosis (DiPALS): a multicentre, open-label, randomised controlled trial. Lancet Neurol (2015) 2.51
A zebrafish compound screen reveals modulation of neutrophil reverse migration as an anti-inflammatory mechanism. Sci Transl Med (2014) 2.51
Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72. Brain (2012) 2.28
Motor neurone disease: a practical update on diagnosis and management. Clin Med (2010) 2.24
L166P mutant DJ-1, causative for recessive Parkinson's disease, is degraded through the ubiquitin-proteasome system. J Biol Chem (2003) 2.12
Systemic delivery of scAAV9 expressing SMN prolongs survival in a model of spinal muscular atrophy. Sci Transl Med (2010) 2.02
The use of non-invasive ventilation at end of life in patients with motor neurone disease: a qualitative exploration of family carer and health professional experiences. Palliat Med (2013) 2.00
Oxidative stress in ALS: key role in motor neuron injury and therapeutic target. Free Radic Biol Med (2009) 1.82
Oxidative stress in ALS: a mechanism of neurodegeneration and a therapeutic target. Biochim Biophys Acta (2006) 1.73
The C9ORF72 expansion mutation is a common cause of ALS+/-FTD in Europe and has a single founder. Eur J Hum Genet (2012) 1.71
Evaluation of human papillomavirus testing for squamous cell carcinoma of the tonsil in clinical practice. J Clin Pathol (2011) 1.70
Microarray analysis of the cellular pathways involved in the adaptation to and progression of motor neuron injury in the SOD1 G93A mouse model of familial ALS. J Neurosci (2007) 1.65
Direct evidence for axonal transport defects in a novel mouse model of mutant spastin-induced hereditary spastic paraplegia (HSP) and human HSP patients. J Neurochem (2009) 1.61
Gene expression profiling in human neurodegenerative disease. Nat Rev Neurol (2012) 1.59
Molecular pathology and genetic advances in amyotrophic lateral sclerosis: an emerging molecular pathway and the significance of glial pathology. Acta Neuropathol (2011) 1.58
The microtubule-severing protein Spastin is essential for axon outgrowth in the zebrafish embryo. Hum Mol Genet (2006) 1.54
Ether phospholipids and glycosylinositolphospholipids are not required for amastigote virulence or for inhibition of macrophage activation by Leishmania major. J Biol Chem (2003) 1.52
Microglia as potential contributors to motor neuron injury in amyotrophic lateral sclerosis. Glia (2005) 1.47
Update on the glutamatergic neurotransmitter system and the role of excitotoxicity in amyotrophic lateral sclerosis. Muscle Nerve (2002) 1.44
Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS. Proc Natl Acad Sci U S A (2013) 1.44
Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis. Brain (2002) 1.43
Using transcutaneous carbon dioxide monitor (TOSCA 500) to detect respiratory failure in patients with amyotrophic lateral sclerosis: a validation study. Amyotroph Lateral Scler (2012) 1.43
Current developments in gene therapy for amyotrophic lateral sclerosis. Expert Opin Biol Ther (2015) 1.43
Cardiopulmonary bypass without ischemic arrest: a myocardial protection strategy for high-risk patients requiring urgent CABG. J Card Surg (2006) 1.37
Unraveling tissue regeneration pathways using chemical genetics. J Biol Chem (2007) 1.35
Review: When is an antioxidant not an antioxidant? A review of novel actions and reactions of vitamin C. Free Radic Res (2005) 1.30
Novel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis. Arch Neurol (2010) 1.29
Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS). PLoS One (2010) 1.28
Rasch analysis of the hospital anxiety and depression scale (HADS) for use in motor neurone disease. Health Qual Life Outcomes (2011) 1.23
Non-invasive ventilation in motor neuron disease: an update of current UK practice. J Neurol Neurosurg Psychiatry (2011) 1.22
The cellular and molecular pathology of the motor system in hereditary spastic paraparesis due to mutation of the spastin gene. J Neuropathol Exp Neurol (2003) 1.21
DNA repair is responsible for the presence of oxidatively damaged DNA lesions in urine. Mutat Res (2005) 1.18
Mitochondrial involvement in amyotrophic lateral sclerosis. Neurochem Int (2002) 1.18
Early interneuron dysfunction in ALS: insights from a mutant sod1 zebrafish model. Ann Neurol (2012) 1.17
Cu/Zn superoxide dismutase (SOD1) mutations associated with familial amyotrophic lateral sclerosis (ALS) affect cellular free radical release in the presence of oxidative stress. Amyotroph Lateral Scler Other Motor Neuron Disord (2002) 1.17
Diagnosis and management of motor neurone disease. BMJ (2008) 1.16
Mechanisms, models and biomarkers in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener (2013) 1.16
Identification of a 3-deoxy-D-manno-octulosonic acid biosynthetic operon in Moraxella catarrhalis and analysis of a KdsA-deficient isogenic mutant. Infect Immun (2003) 1.13
Sphingosine-1-phosphate mediates transcriptional regulation of key targets associated with survival, proliferation, and pluripotency in human embryonic stem cells. Stem Cells Dev (2008) 1.13
The widening spectrum of C9ORF72-related disease; genotype/phenotype correlations and potential modifiers of clinical phenotype. Acta Neuropathol (2014) 1.13
Role of lgtC in resistance of nontypeable Haemophilus influenzae strain R2866 to human serum. Infect Immun (2006) 1.12
Dysregulation of astrocyte-motoneuron cross-talk in mutant superoxide dismutase 1-related amyotrophic lateral sclerosis. Brain (2011) 1.11
Selective and signal-dependent recruitment of membrane proteins to secretory granules formed by heterologously expressed von Willebrand factor. Mol Biol Cell (2002) 1.11
Hereditary spastic paraparesis: disrupted intracellular transport associated with spastin mutation. Ann Neurol (2003) 1.11
Regulation of sialic acid transport and catabolism in Haemophilus influenzae. Mol Microbiol (2007) 1.10
Bacterial social networks: structure and composition of Myxococcus xanthus outer membrane vesicle chains. Environ Microbiol (2013) 1.09
Astrocyte function and role in motor neuron disease: a future therapeutic target? Glia (2009) 1.09
TMEM106B is a genetic modifier of frontotemporal lobar degeneration with C9orf72 hexanucleotide repeat expansions. Acta Neuropathol (2014) 1.08
Characterization of the N-acetyl-5-neuraminic acid-binding site of the extracytoplasmic solute receptor (SiaP) of nontypeable Haemophilus influenzae strain 2019. J Biol Chem (2007) 1.08
Microarray analysis of the astrocyte transcriptome in the aging brain: relationship to Alzheimer's pathology and APOE genotype. Neurobiol Aging (2011) 1.05
Review of fluorescent standards for calibration of in situ fluorometers: recommendations applied in coastal and ocean observing programs. Opt Express (2011) 1.05
Microarray RNA expression analysis of cerebral white matter lesions reveals changes in multiple functional pathways. Stroke (2008) 1.05
Unravelling the enigma of selective vulnerability in neurodegeneration: motor neurons resistant to degeneration in ALS show distinct gene expression characteristics and decreased susceptibility to excitotoxicity. Acta Neuropathol (2012) 1.05
Expression of vascular endothelial growth factor and its receptors in the central nervous system in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol (2006) 1.04
ALS-associated mutations in FUS disrupt the axonal distribution and function of SMN. Hum Mol Genet (2013) 1.04
Acid-catalyzed oxygen-18 labeling of peptides. Anal Chem (2009) 1.02
Pattern of spread and prognosis in lower limb-onset ALS. Amyotroph Lateral Scler (2010) 1.02
Neuronal dark matter: the emerging role of microRNAs in neurodegeneration. Front Cell Neurosci (2013) 1.02
Modeling inflammation in the zebrafish: how a fish can help us understand lung disease. Exp Lung Res (2007) 1.02
Broad clinical phenotypes associated with TAR-DNA binding protein (TARDBP) mutations in amyotrophic lateral sclerosis. Neurogenetics (2009) 1.02
Design, implementation and multisite evaluation of a system suitability protocol for the quantitative assessment of instrument performance in liquid chromatography-multiple reaction monitoring-MS (LC-MRM-MS). Mol Cell Proteomics (2013) 1.01
Simultaneous and independent detection of C9ORF72 alleles with low and high number of GGGGCC repeats using an optimised protocol of Southern blot hybridisation. Mol Neurodegener (2013) 1.01
Lectin chromatography/mass spectrometry discovery workflow identifies putative biomarkers of aggressive breast cancers. J Proteome Res (2012) 1.01
Zebrafish as a model for the study of neutrophil biology. J Leukoc Biol (2013) 1.01
Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS). PLoS One (2011) 1.00
Apoptosis in amyotrophic lateral sclerosis--what is the evidence? Lancet Neurol (2005) 1.00
Phosphatase and tensin homologue/protein kinase B pathway linked to motor neuron survival in human superoxide dismutase 1-related amyotrophic lateral sclerosis. Brain (2011) 1.00
Microarray analysis of murine palatogenesis: temporal expression of genes during normal palate development. Dev Growth Differ (2003) 1.00
Alpha-synuclein mRNA expression in oligodendrocytes in MSA. Glia (2014) 0.99
Large-scale pathways-based association study in amyotrophic lateral sclerosis. Brain (2007) 0.98
A genome-wide association meta-analysis identifies a novel locus at 17q11.2 associated with sporadic amyotrophic lateral sclerosis. Hum Mol Genet (2013) 0.98
Motor neuron disease in a patient with a mitochondrial tRNAIle mutation. Ann Neurol (2006) 0.98
Alterations in the blood brain barrier in ageing cerebral cortex in relationship to Alzheimer-type pathology: a study in the MRC-CFAS population neuropathology cohort. Neurosci Lett (2011) 0.98
Production of monocyte chemoattractant protein-1 in amyotrophic lateral sclerosis. Muscle Nerve (2005) 0.97