Published in Nat Neurosci on April 01, 2002
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Aggregation propensities of superoxide dismutase G93 hotspot mutants mirror ALS clinical phenotypes. Proc Natl Acad Sci U S A (2014) 0.80
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The Copper Metabolism MURR1 domain protein 1 (COMMD1) modulates the aggregation of misfolded protein species in a client-specific manner. PLoS One (2014) 0.78
Redox environment is an intracellular factor to operate distinct pathways for aggregation of Cu,Zn-superoxide dismutase in amyotrophic lateral sclerosis. Front Cell Neurosci (2013) 0.78
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A faulty interaction between SOD1 and hCCS in neurodegenerative disease. Sci Rep (2016) 0.76
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A Copper Binding Site within the Pathological Conformer Epitope of Mutant SOD1. Front Neurosci (2011) 0.75
A proposed mechanism of ALS fails the test in vivo. Nat Neurosci (2002) 0.75
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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
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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
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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
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Centromere-specific assembly of CENP-a nucleosomes is mediated by HJURP. Cell (2009) 4.22
A test of climate, sun, and culture relationships from an 1810-year Chinese cave record. Science (2008) 3.94
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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
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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
BACE1, a major determinant of selective vulnerability of the brain to amyloid-beta amyloidogenesis, is essential for cognitive, emotional, and synaptic functions. J Neurosci (2005) 3.23
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
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
Association of gamma-secretase with lipid rafts in post-Golgi and endosome membranes. J Biol Chem (2004) 2.85
Parkinson's disease alpha-synuclein transgenic mice develop neuronal mitochondrial degeneration and cell death. J Neurosci (2006) 2.85
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The beta-secretase enzyme BACE in health and Alzheimer's disease: regulation, cell biology, function, and therapeutic potential. J Neurosci (2009) 2.80
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Chemoenzymatic synthesis of homogeneous ultralow molecular weight heparins. Science (2011) 2.79
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
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Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Curr Opin Neurobiol (2011) 2.48
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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
Aggregation promoting C-terminal truncation of alpha-synuclein is a normal cellular process and is enhanced by the familial Parkinson's disease-linked mutations. Proc Natl Acad Sci U S A (2005) 2.45
An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study. Lancet Neurol (2013) 2.43
ZW10 links mitotic checkpoint signaling to the structural kinetochore. J Cell Biol (2005) 2.36
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Clonal vaccinia virus grown in cell culture as a new smallpox vaccine. Nat Med (2003) 2.27
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An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS. Nat Med (2007) 2.20
Using an enzymatic combinatorial approach to identify anticoagulant heparan sulfate structures. Chem Biol (2007) 2.20
Atp7a determines a hierarchy of copper metabolism essential for notochord development. Cell Metab (2006) 2.14
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
Catalytic assembly of the mitotic checkpoint inhibitor BubR1-Cdc20 by a Mad2-induced functional switch in Cdc20. Mol Cell (2013) 2.06
Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism. Proc Natl Acad Sci U S A (2010) 2.05
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Axonal transport, amyloid precursor protein, kinesin-1, and the processing apparatus: revisited. J Neurosci (2005) 2.04
The seeds of neurodegeneration: prion-like spreading in ALS. Cell (2011) 2.03
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Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A. J Cell Biol (2003) 2.02
Mast cells modulate the pathogenesis of elastase-induced abdominal aortic aneurysms in mice. J Clin Invest (2007) 2.00
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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
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A two-stage genome-wide association study of sporadic amyotrophic lateral sclerosis. Hum Mol Genet (2009) 1.93
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Enzymatic redesigning of biologically active heparan sulfate. J Biol Chem (2005) 1.89
NPM-ALK transgenic mice spontaneously develop T-cell lymphomas and plasma cell tumors. Blood (2002) 1.87
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
High-throughput microarray detection of olfactory receptor gene expression in the mouse. Proc Natl Acad Sci U S A (2004) 1.82
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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