Published in Proc Natl Acad Sci U S A on August 22, 2016
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature (1993) 21.65
ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron (2006) 7.82
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron (1997) 6.16
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol (2009) 5.65
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nat Neurosci (2010) 4.25
Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria. Proc Natl Acad Sci U S A (2006) 3.30
Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells. Proc Natl Acad Sci U S A (2011) 3.23
Targeted disruption of migration inhibitory factor gene reveals its critical role in sepsis. J Exp Med (1999) 3.23
Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron (2004) 2.94
Oxidized/misfolded superoxide dismutase-1: the cause of all amyotrophic lateral sclerosis? Ann Neurol (2007) 2.76
Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS. Prog Neurobiol (2008) 2.76
Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J Biol Chem (2002) 2.68
Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Curr Opin Neurobiol (2011) 2.48
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1. Genes Dev (2008) 2.43
Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria. Proc Natl Acad Sci U S A (2008) 2.35
An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS. Nat Med (2007) 2.20
Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patients. PLoS One (2010) 1.89
Intermolecular transmission of superoxide dismutase 1 misfolding in living cells. Proc Natl Acad Sci U S A (2011) 1.82
Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice. J Neurosci (2005) 1.79
Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron (2010) 1.65
A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis. J Biol Chem (2008) 1.65
Macrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1. Neuron (2015) 1.57
Disulfide analysis reveals a role for macrophage migration inhibitory factor (MIF) as thiol-protein oxidoreductase. J Mol Biol (1998) 1.56
A novel monoclonal antibody reveals a conformational alteration shared by amyotrophic lateral sclerosis-linked SOD1 mutants. Ann Neurol (2012) 1.55
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
Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form. Acta Neuropathol (2010) 1.49
Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms. Proc Natl Acad Sci U S A (2014) 1.48
Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS. J Neurochem (2010) 1.47
An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1. Proc Natl Acad Sci U S A (2012) 1.45
Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis. PLoS One (2012) 1.42
ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2. Hum Mol Genet (2010) 1.31
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
The Golgi-associated protein p115 mediates the secretion of macrophage migration inhibitory factor. J Immunol (2009) 1.25
Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models. J Neurosci (2006) 1.18
Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS. Proc Natl Acad Sci U S A (2012) 1.17
Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis. Ann Neurol (2009) 1.17
Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis. J Neurochem (2005) 1.16
Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis. Acta Neuropathol (2011) 1.13
Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression. Neurobiol Dis (2007) 1.11
Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. PLoS One (2011) 1.07
Null mutation in macrophage migration inhibitory factor prevents muscle cell loss and fibrosis in partial bladder outlet obstruction. Am J Physiol Renal Physiol (2006) 0.97
Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1. Hum Mol Genet (2013) 0.96
An examination of alpha B-crystallin as a modifier of SOD1 aggregate pathology and toxicity in models of familial amyotrophic lateral sclerosis. J Neurochem (2010) 0.94
Misfolded superoxide dismutase-1 in CSF from amyotrophic lateral sclerosis patients. J Neurochem (2011) 0.93
Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mouse model of amyotrophic lateral sclerosis. J Neurochem (2008) 0.91
Prion-like activity of Cu/Zn superoxide dismutase: implications for amyotrophic lateral sclerosis. Prion (2014) 0.89
Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases. Acta Neuropathol Commun (2014) 0.89
Chaperone-like activity of macrophage migration inhibitory factor. Int J Biochem Cell Biol (2005) 0.88
Derlin-1 overexpression ameliorates mutant SOD1-induced endoplasmic reticulum stress by reducing mutant SOD1 accumulation. Neurochem Int (2010) 0.88
SOD1 as a molecular switch for initiating the homeostatic ER stress response under zinc deficiency. Mol Cell (2013) 0.86
Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110. Proc Natl Acad Sci U S A (2016) 0.81
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