Published in J Neurochem on May 01, 2005
Antisense oligonucleotide therapy for neurodegenerative disease. J Clin Invest (2006) 3.00
Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons. Proc Natl Acad Sci U S A (2007) 1.79
Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis. J Neurosci (2013) 1.51
A drosophila model for amyotrophic lateral sclerosis reveals motor neuron damage by human SOD1. J Biol Chem (2008) 1.23
Characterization of the properties of a novel mutation in VAPB in familial amyotrophic lateral sclerosis. J Biol Chem (2010) 1.19
Altered axonal architecture by removal of the heavily phosphorylated neurofilament tail domains strongly slows superoxide dismutase 1 mutant-mediated ALS. Proc Natl Acad Sci U S A (2005) 1.19
Blocking the mitochondrial apoptotic pathway preserves motor neuron viability and function in a mouse model of amyotrophic lateral sclerosis. J Clin Invest (2010) 1.11
Characterization of detergent-insoluble proteins in ALS indicates a causal link between nitrative stress and aggregation in pathogenesis. PLoS One (2009) 1.10
Pre-symptomatic detection of chronic motor deficits and genotype prediction in congenic B6.SOD1(G93A) ALS mouse model. Neuroscience (2009) 1.00
Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases. Neurobiol Aging (2007) 1.00
Proteins that bind to misfolded mutant superoxide dismutase-1 in spinal cords from transgenic amyotrophic lateral sclerosis (ALS) model mice. J Biol Chem (2011) 0.94
Molecular chaperones and neuronal proteostasis. Semin Cell Dev Biol (2015) 0.91
Molecular chaperone mediated late-stage neuroprotection in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis. PLoS One (2013) 0.91
Protein Quality Control by Molecular Chaperones in Neurodegeneration. Front Neurosci (2017) 0.87
The in vivo contribution of motor neuron TrkB receptors to mutant SOD1 motor neuron disease. Hum Mol Genet (2011) 0.84
SIRT1 overexpression ameliorates a mouse model of SOD1-linked amyotrophic lateral sclerosis via HSF1/HSP70i chaperone system. Mol Brain (2014) 0.82
Chaperone-dependent Neurodegeneration: A Molecular Perspective on Therapeutic Intervention. J Alzheimers Dis Parkinsonism (2013) 0.81
Administration of Recombinant Heat Shock Protein 70 Delays Peripheral Muscle Denervation in the SOD1(G93A) Mouse Model of Amyotrophic Lateral Sclerosis. Neurol Res Int (2012) 0.80
Effect of CCS on the accumulation of FALS SOD1 mutant-containing aggregates and on mitochondrial translocation of SOD1 mutants: implication of a free radical hypothesis. Arch Biochem Biophys (2011) 0.80
Induction of heat shock proteins in differentiated human neuronal cells following co-application of celastrol and arimoclomol. Cell Stress Chaperones (2016) 0.78
ZNStress: a high-throughput drug screening protocol for identification of compounds modulating neuronal stress in the transgenic mutant sod1G93R zebrafish model of amyotrophic lateral sclerosis. Mol Neurodegener (2016) 0.78
Amyotrophic lateral sclerosis: Protein chaperone dysfunction revealed by proteomic studies of animal models. Proteomics Clin Appl (2008) 0.76
Substantially elevating the levels of αB-crystallin in spinal motor neurons of mutant SOD1 mice does not significantly delay paralysis or attenuate mutant protein aggregation. J Neurochem (2015) 0.76
Huntingtin fragments and SOD1 mutants form soluble oligomers in the cell. PLoS One (2012) 0.76
Stressing Out Hsp90 in Neurotoxic Proteinopathies. Curr Top Med Chem (2016) 0.75
Endogenous macrophage migration inhibitory factor reduces the accumulation and toxicity of misfolded SOD1 in a mouse model of ALS. Proc Natl Acad Sci U S A (2016) 0.75
The heat shock response in neurons and astroglia and its role in neurodegenerative diseases. Mol Neurodegener (2017) 0.75
Onset and progression in inherited ALS determined by motor neurons and microglia. Science (2006) 9.42
ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron (2006) 7.82
On the road to cancer: aneuploidy and the mitotic checkpoint. Nat Rev Cancer (2005) 7.53
Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu Rev Neurosci (2004) 6.99
The human CENP-A centromeric nucleosome-associated complex. Nat Cell Biol (2006) 6.85
Boveri revisited: chromosomal instability, aneuploidy and tumorigenesis. Nat Rev Mol Cell Biol (2009) 6.43
Aneuploidy acts both oncogenically and as a tumor suppressor. Cancer Cell (2006) 6.37
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. Nat Neurosci (2011) 6.01
Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci (2008) 5.89
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet (2010) 5.75
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol (2009) 5.65
Rethinking ALS: the FUS about TDP-43. Cell (2009) 4.71
Propagation of centromeric chromatin requires exit from mitosis. J Cell Biol (2007) 4.60
Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis. Neuron (2012) 4.48
A standardized kinesin nomenclature. J Cell Biol (2004) 4.43
Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc Natl Acad Sci U S A (2002) 4.37
Structural determinants for generating centromeric chromatin. Nature (2004) 4.25
Centromere-specific assembly of CENP-a nucleosomes is mediated by HJURP. Cell (2009) 4.22
Does aneuploidy cause cancer? Curr Opin Cell Biol (2006) 3.89
ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci (2008) 3.78
Decoding the links between mitosis, cancer, and chemotherapy: The mitotic checkpoint, adaptation, and cell death. Cancer Cell (2005) 3.69
Lethality to human cancer cells through massive chromosome loss by inhibition of the mitotic checkpoint. Proc Natl Acad Sci U S A (2004) 3.69
Dynamics of centromere and kinetochore proteins; implications for checkpoint signaling and silencing. Curr Biol (2004) 3.65
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63
Unattached kinetochores catalyze production of an anaphase inhibitor that requires a Mad2 template to prime Cdc20 for BubR1 binding. Dev Cell (2009) 3.36
Motoneuron death triggered by a specific pathway downstream of Fas. potentiation by ALS-linked SOD1 mutations. Neuron (2002) 3.31
Unstable kinetochore-microtubule capture and chromosomal instability following deletion of CENP-E. Dev Cell (2002) 3.23
Centromere-associated protein-E is essential for the mammalian mitotic checkpoint to prevent aneuploidy due to single chromosome loss. J Cell Biol (2003) 3.15
Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading. Nat Neurosci (2002) 3.09
Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease. Nat Neurosci (2007) 3.02
Antisense oligonucleotide therapy for neurodegenerative disease. J Clin Invest (2006) 3.00
Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration. Proc Natl Acad Sci U S A (2013) 2.95
Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron (2004) 2.94
Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs. Nat Neurosci (2012) 2.93
Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression. Cell (2012) 2.83
Centromere identity maintained by nucleosomes assembled with histone H3 containing the CENP-A targeting domain. Mol Cell (2007) 2.76
Epigenetic centromere propagation and the nature of CENP-a nucleosomes. Cell (2011) 2.74
Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis. Nat Neurosci (2013) 2.71
ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS. Proc Natl Acad Sci U S A (2010) 2.65
Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Curr Opin Neurobiol (2011) 2.48
Activating and silencing the mitotic checkpoint through CENP-E-dependent activation/inactivation of BubR1. Cell (2003) 2.47
Cep152 interacts with Plk4 and is required for centriole duplication. J Cell Biol (2010) 2.45
ZW10 links mitotic checkpoint signaling to the structural kinetochore. J Cell Biol (2005) 2.36
Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria. Proc Natl Acad Sci U S A (2008) 2.35
Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2007) 2.25
An epigenetic mark generated by the incorporation of CENP-A into centromeric nucleosomes. Proc Natl Acad Sci U S A (2007) 2.23
Evaluation of non-invasive prenatal RHD genotyping of the fetus. Med J Aust (2009) 2.21
An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS. Nat Med (2007) 2.20
Aneuploidy: instigator and inhibitor of tumorigenesis. Cancer Res (2007) 2.12
Microtubule capture by CENP-E silences BubR1-dependent mitotic checkpoint signaling. J Cell Biol (2005) 2.11
Epithelial-mesenchymal transition events during human embryonic stem cell differentiation. Cancer Res (2007) 2.10
Catalytic assembly of the mitotic checkpoint inhibitor BubR1-Cdc20 by a Mad2-induced functional switch in Cdc20. Mol Cell (2013) 2.06
Polo-like kinase 4 kinase activity limits centrosome overduplication by autoregulating its own stability. J Cell Biol (2010) 2.05
The seeds of neurodegeneration: prion-like spreading in ALS. Cell (2011) 2.03
Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A. J Cell Biol (2003) 2.02
Aurora kinases and protein phosphatase 1 mediate chromosome congression through regulation of CENP-E. Cell (2010) 2.00
Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2006) 1.97
CENP-A-containing nucleosomes: easier disassembly versus exclusive centromeric localization. J Mol Biol (2007) 1.93
Elevated PGC-1α activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS. Cell Metab (2012) 1.92
Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells. J Clin Invest (2009) 1.86
Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice. Proc Natl Acad Sci U S A (2008) 1.84
Losing balance: the origin and impact of aneuploidy in cancer. EMBO Rep (2012) 1.81
Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons. Proc Natl Acad Sci U S A (2007) 1.79
CENP-E combines a slow, processive motor and a flexible coiled coil to produce an essential motile kinetochore tether. J Cell Biol (2008) 1.77
Removal of Spindly from microtubule-attached kinetochores controls spindle checkpoint silencing in human cells. Genes Dev (2010) 1.66
Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron (2010) 1.65
Abrogation of E-cadherin-mediated cell-cell contact in mouse embryonic stem cells results in reversible LIF-independent self-renewal. Stem Cells (2009) 1.63
A two-step mechanism for epigenetic specification of centromere identity and function. Nat Cell Biol (2013) 1.52
Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis. J Neurosci (2013) 1.51
Medicine. Treating neurodegenerative diseases with antibiotics. Science (2005) 1.51
Prion-like spread of protein aggregates in neurodegeneration. J Exp Med (2012) 1.49
Unstable microtubule capture at kinetochores depleted of the centromere-associated protein CENP-F. EMBO J (2005) 1.48
NuMA after 30 years: the matrix revisited. Trends Cell Biol (2010) 1.48
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. Proc Natl Acad Sci U S A (2013) 1.48
Double-strand DNA breaks recruit the centromeric histone CENP-A. Proc Natl Acad Sci U S A (2009) 1.47
Requirements for NuMA in maintenance and establishment of mammalian spindle poles. J Cell Biol (2009) 1.47
Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc Natl Acad Sci U S A (2008) 1.45
Human Zwint-1 specifies localization of Zeste White 10 to kinetochores and is essential for mitotic checkpoint signaling. J Biol Chem (2004) 1.44
Virus-delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis. Ann Neurol (2005) 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
Chromosomal instability by inefficient Mps1 auto-activation due to a weakened mitotic checkpoint and lagging chromosomes. PLoS One (2008) 1.43
Misregulated RNA processing in amyotrophic lateral sclerosis. Brain Res (2012) 1.42
NF-M is an essential target for the myelin-directed "outside-in" signaling cascade that mediates radial axonal growth. J Cell Biol (2003) 1.41
The role of aneuploidy in promoting and suppressing tumors. J Cell Biol (2009) 1.39
Therapeutic AAV9-mediated suppression of mutant SOD1 slows disease progression and extends survival in models of inherited ALS. Mol Ther (2013) 1.36
Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS. PLoS One (2008) 1.34
Inducible, reversible system for the rapid and complete degradation of proteins in mammalian cells. Proc Natl Acad Sci U S A (2012) 1.33
Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice. Proc Natl Acad Sci U S A (2009) 1.33
Chromoanagenesis and cancer: mechanisms and consequences of localized, complex chromosomal rearrangements. Nat Med (2012) 1.32
Chromosome missegregation rate predicts whether aneuploidy will promote or suppress tumors. Proc Natl Acad Sci U S A (2013) 1.28
Human condensin function is essential for centromeric chromatin assembly and proper sister kinetochore orientation. PLoS One (2009) 1.27
ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import. Proc Natl Acad Sci U S A (2010) 1.26
Sox2 is essential for formation of trophectoderm in the preimplantation embryo. PLoS One (2010) 1.26
E-cadherin inhibits cell surface localization of the pro-migratory 5T4 oncofetal antigen in mouse embryonic stem cells. Mol Biol Cell (2007) 1.26
A chemical tool box defines mitotic and interphase roles for Mps1 kinase. J Cell Biol (2010) 1.25
Essential alterations of heparan sulfate during the differentiation of embryonic stem cells to Sox1-enhanced green fluorescent protein-expressing neural progenitor cells. Stem Cells (2007) 1.23
Error-prone mammalian female meiosis from silencing the spindle assembly checkpoint without normal interkinetochore tension. Proc Natl Acad Sci U S A (2012) 1.23