Published in Neuron on July 08, 2004
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A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice. Nat Neurosci (2009) 3.89
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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
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SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse. Brain (2010) 1.21
Complex genetics of amyotrophic lateral sclerosis. Am J Hum Genet (2004) 1.20
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
VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasis. Hum Mol Genet (2011) 1.19
Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons. J Neurosci (2012) 1.19
Impaired post-translational folding of familial ALS-linked Cu, Zn superoxide dismutase mutants. EMBO J (2007) 1.18
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Mitochondrial proteomic analysis of a cell line model of familial amyotrophic lateral sclerosis. Mol Cell Proteomics (2004) 1.17
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S-nitrosothiol depletion in amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2006) 1.11
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
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Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. PLoS One (2011) 1.07
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Inhibition of mitochondrial protein import by mutant huntingtin. Nat Neurosci (2014) 1.04
The ER mitochondria calcium cycle and ER stress response as therapeutic targets in amyotrophic lateral sclerosis. Front Cell Neurosci (2014) 1.02
Metal deficiency increases aberrant hydrophobicity of mutant superoxide dismutases that cause amyotrophic lateral sclerosis. J Biol Chem (2009) 1.02
Disulfide-reduced ALS variants of Cu, Zn superoxide dismutase exhibit increased populations of unfolded species. J Mol Biol (2010) 1.01
A method for probing the mutational landscape of amyloid structure. Bioinformatics (2011) 1.01
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The mitochondrial permeability transition pore: a molecular target for amyotrophic lateral sclerosis therapy. Biochim Biophys Acta (2009) 0.98
Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria. J Biol Chem (2008) 0.98
Oral administration of a small molecule targeted to block proNGF binding to p75 promotes myelin sparing and functional recovery after spinal cord injury. J Neurosci (2013) 0.98
Mitochondria in neurodegeneration. Adv Exp Med Biol (2012) 0.97
Increased axonal mitochondrial mobility does not slow amyotrophic lateral sclerosis (ALS)-like disease in mutant SOD1 mice. J Biol Chem (2011) 0.97
Opposite regulation of oligodendrocyte apoptosis by JNK3 and Pin1 after spinal cord injury. J Neurosci (2007) 0.97
Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1. Hum Mol Genet (2013) 0.96
Stem cell-derived motor neurons: applications and challenges in amyotrophic lateral sclerosis. Curr Stem Cell Res Ther (2009) 0.95
Defective mitochondrial dynamics is an early event in skeletal muscle of an amyotrophic lateral sclerosis mouse model. PLoS One (2013) 0.94
A role for sequestosome 1/p62 in mitochondrial dynamics, import and genome integrity. Biochim Biophys Acta (2012) 0.94
Decreased glutathione accelerates neurological deficit and mitochondrial pathology in familial ALS-linked hSOD1(G93A) mice model. Neurobiol Dis (2011) 0.94
Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein. J Biol Chem (2008) 0.94
A comprehensive review of amyotrophic lateral sclerosis. Surg Neurol Int (2015) 0.93
Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS. Proc Natl Acad Sci U S A (2015) 0.93
Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis. Biochim Biophys Acta (2014) 0.92
Bcl-2 is a novel interacting partner for the 2-oxoglutarate carrier and a key regulator of mitochondrial glutathione. Free Radic Biol Med (2011) 0.92
A fruitful endeavor: modeling ALS in the fruit fly. Brain Res (2014) 0.91
Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo. Neurobiol Dis (2009) 0.91
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Calcium homeostasis in aging neurons. Front Genet (2012) 0.86
Calcium dysregulation links ALS defective proteins and motor neuron selective vulnerability. Front Cell Neurosci (2015) 0.84
Inaccurately assembled cytochrome c oxidase can lead to oxidative stress-induced growth arrest. Antioxid Redox Signal (2013) 0.84
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis. Nat Genet (2008) 9.51
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ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron (2006) 7.82
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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
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
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Axonopathy and transport deficits early in the pathogenesis of Alzheimer's disease. Science (2005) 5.49
The centrosomal protein nephrocystin-6 is mutated in Joubert syndrome and activates transcription factor ATF4. Nat Genet (2006) 5.36
Rethinking ALS: the FUS about TDP-43. Cell (2009) 4.71
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A standardized kinesin nomenclature. J Cell Biol (2004) 4.43
Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis. Nat Genet (2009) 4.38
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
VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death. Nat Genet (2003) 4.19
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A (2009) 4.18
Candidate exome capture identifies mutation of SDCCAG8 as the cause of a retinal-renal ciliopathy. Nat Genet (2010) 3.99
In-frame deletion in a novel centrosomal/ciliary protein CEP290/NPHP6 perturbs its interaction with RPGR and results in early-onset retinal degeneration in the rd16 mouse. Hum Mol Genet (2006) 3.90
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ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis. Nat Genet (2006) 3.88
ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci (2008) 3.78
Cadherin 23 is a component of the tip link in hair-cell stereocilia. Nature (2004) 3.69
Decoding the links between mitosis, cancer, and chemotherapy: The mitotic checkpoint, adaptation, and cell death. Cancer Cell (2005) 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
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The power of social networking in medicine. Nat Biotechnol (2009) 2.37
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