Published in J Neurosci Methods on March 15, 2002
Molecular and cellular pathways of neurodegeneration in motor neurone disease. J Neurol Neurosurg Psychiatry (2005) 1.55
ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2. Hum Mol Genet (2010) 1.31
Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities. Hum Mol Genet (2009) 1.30
Mitochondrial dysfunction in amyotrophic lateral sclerosis. Biochim Biophys Acta (2009) 1.27
Peroxisome proliferator activator receptor gamma coactivator-1alpha (PGC-1α) improves motor performance and survival in a mouse model of amyotrophic lateral sclerosis. Mol Neurodegener (2011) 1.23
Complex genetics of amyotrophic lateral sclerosis. Am J Hum Genet (2004) 1.20
SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments. Mamm Genome (2011) 1.17
Neuroprotective effects of near-infrared light in an in vivo model of mitochondrial optic neuropathy. J Neurosci (2008) 1.13
The Psi(m) depolarization that accompanies mitochondrial Ca2+ uptake is greater in mutant SOD1 than in wild-type mouse motor terminals. Proc Natl Acad Sci U S A (2009) 1.11
The complex molecular biology of amyotrophic lateral sclerosis (ALS). Prog Mol Biol Transl Sci (2012) 1.05
Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria. J Biol Chem (2008) 0.98
Mitochondria in neurodegeneration. Adv Exp Med Biol (2012) 0.97
The small GTPase Arf1 modulates mitochondrial morphology and function. EMBO J (2014) 0.94
Caprylic triglyceride as a novel therapeutic approach to effectively improve the performance and attenuate the symptoms due to the motor neuron loss in ALS disease. PLoS One (2012) 0.93
Measures of bulbar and spinal motor function, muscle innervation, and mitochondrial function in ALS rats. Behav Brain Res (2010) 0.90
Striatal neuroprotection with methylene blue. Neuroscience (2009) 0.90
Neuroprotective effects of memantine in a mouse model of retinal degeneration induced by rotenone. Brain Res (2008) 0.84
A tissue-specific approach to the analysis of metabolic changes in Caenorhabditis elegans. PLoS One (2011) 0.83
Mitochondria-targeted catalase reverts the neurotoxicity of hSOD1G⁹³A astrocytes without extending the survival of ALS-linked mutant hSOD1 mice. PLoS One (2014) 0.82
Respiratory chain deficiency in aged spinal motor neurons. Neurobiol Aging (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
Mouse models of mitochondrial complex I dysfunction. Int J Biochem Cell Biol (2012) 0.77
Dysregulated expression of death, stress and mitochondrion related genes in the sciatic nerve of presymptomatic SOD1(G93A) mouse model of Amyotrophic Lateral Sclerosis. Front Cell Neurosci (2015) 0.75
A high-fat jelly diet restores bioenergetic balance and extends lifespan in the presence of motor dysfunction and lumbar spinal cord motor neuron loss in TDP-43A315T mutant C57BL6/J mice. Dis Model Mech (2016) 0.75
Asymmetry in the assembly of the RNAi enzyme complex. Cell (2003) 22.18
Designing siRNA that distinguish between genes that differ by a single nucleotide. PLoS Genet (2006) 3.23
Mitochondrial dysfunction and its role in motor neuron degeneration in ALS. Mitochondrion (2005) 2.24
Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model. Proc Natl Acad Sci U S A (2006) 2.10
Several rAAV vectors efficiently cross the blood-brain barrier and transduce neurons and astrocytes in the neonatal mouse central nervous system. Mol Ther (2011) 1.96
Selective silencing by RNAi of a dominant allele that causes amyotrophic lateral sclerosis. Aging Cell (2003) 1.88
An RNA polymerase II construct synthesizes short-hairpin RNA with a quantitative indicator and mediates highly efficient RNAi. Nucleic Acids Res (2005) 1.83
Reactive astrocytes secrete lcn2 to promote neuron death. Proc Natl Acad Sci U S A (2013) 1.59
Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS. J Neurosci (2002) 1.55
Pol II-expressed shRNA knocks down Sod2 gene expression and causes phenotypes of the gene knockout in mice. PLoS Genet (2006) 1.49
ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes. BMC Neurosci (2003) 1.46
The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanism. PLoS One (2010) 1.32
Aberrantly increased hydrophobicity shared by mutants of Cu,Zn-superoxide dismutase in familial amyotrophic lateral sclerosis. J Biol Chem (2005) 1.30
Allele-specific RNAi selectively silences mutant SOD1 and achieves significant therapeutic benefit in vivo. Neurobiol Dis (2006) 1.30
Mitochondrial electron transport chain complex dysfunction in a transgenic mouse model for amyotrophic lateral sclerosis. J Neurochem (2002) 1.27
An enhanced U6 promoter for synthesis of short hairpin RNA. Nucleic Acids Res (2003) 1.24
Multiple shRNAs expressed by an inducible pol II promoter can knock down the expression of multiple target genes. Biotechniques (2006) 1.21
Silencing of the Pink1 gene expression by conditional RNAi does not induce dopaminergic neuron death in mice. Int J Biol Sci (2007) 1.20
S-nitrosothiol depletion in amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2006) 1.11
A construct with fluorescent indicators for conditional expression of miRNA. BMC Biotechnol (2008) 1.08
Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2014) 1.08
Widespread spinal cord transduction by intrathecal injection of rAAV delivers efficacious RNAi therapy for amyotrophic lateral sclerosis. Hum Mol Genet (2013) 1.02
Therapeutic gene silencing delivered by a chemically modified small interfering RNA against mutant SOD1 slows amyotrophic lateral sclerosis progression. J Biol Chem (2008) 0.96
Promises and challenges in developing RNAi as a research tool and therapy for neurodegenerative diseases. Neurodegener Dis (2005) 0.93
An RNAi strategy for treatment of amyotrophic lateral sclerosis caused by mutant Cu,Zn superoxide dismutase. J Neurochem (2005) 0.92
Inhibition of chaperone activity is a shared property of several Cu,Zn-superoxide dismutase mutants that cause amyotrophic lateral sclerosis. J Biol Chem (2005) 0.92
SMN protects cells against mutant SOD1 toxicity by increasing chaperone activity. Biochem Biophys Res Commun (2007) 0.90
A non-specific effect associated with conditional transgene expression based on Cre-loxP strategy in mice. PLoS One (2011) 0.86
Widespread aggregation of mutant VAPB associated with ALS does not cause motor neuron degeneration or modulate mutant SOD1 aggregation and toxicity in mice. Mol Neurodegener (2013) 0.85
Deficits in the mitochondrial enzyme α-ketoglutarate dehydrogenase lead to Alzheimer's disease-like calcium dysregulation. Neurobiol Aging (2011) 0.85
Normal dendritic arborization in spinal motoneurons requires neurofilament subunit L. J Comp Neurol (2002) 0.84
Transgenic RNAi: Accelerating and expanding reverse genetics in mammals. Transgenic Res (2006) 0.81
Oxidative stress and autophagic alteration in brainstem of SOD1-G93A mouse model of ALS. Mol Neurobiol (2014) 0.81
Nerve injection of viral vectors efficiently transfers transgenes into motor neurons and delivers RNAi therapy against ALS. Antioxid Redox Signal (2009) 0.79
Specific gene silencing using RNAi in cell culture. Methods Mol Biol (2011) 0.79
Identification of human monoclonal antibodies specific for human SOD1 recognizing distinct epitopes and forms of SOD1. PLoS One (2013) 0.78