Published in J Biol Chem on February 03, 2005
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
Activation of superoxide dismutases: putting the metal to the pedal. Biochim Biophys Acta (2006) 2.33
Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2007) 2.25
Disulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model mice. Proc Natl Acad Sci U S A (2006) 2.22
Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models. Proc Natl Acad Sci U S A (2007) 1.95
Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis. J Biol Chem (2008) 1.69
Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials. Proc Natl Acad Sci U S A (2006) 1.67
Destabilization of apoprotein is insufficient to explain Cu,Zn-superoxide dismutase-linked ALS pathogenesis. Proc Natl Acad Sci U S A (2005) 1.67
Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival. PLoS Biol (2008) 1.61
Mapping superoxide dismutase 1 domains of non-native interaction: roles of intra- and intermolecular disulfide bonding in aggregation. J Neurochem (2006) 1.59
Extracellular mutant SOD1 induces microglial-mediated motoneuron injury. Glia (2010) 1.54
Aggregation of copper-zinc superoxide dismutase in familial and sporadic ALS. Antioxid Redox Signal (2009) 1.52
Modifications of superoxide dismutase (SOD1) in human erythrocytes: a possible role in amyotrophic lateral sclerosis. J Biol Chem (2009) 1.46
Cysteine 111 affects aggregation and cytotoxicity of mutant Cu,Zn-superoxide dismutase associated with familial amyotrophic lateral sclerosis. J Biol Chem (2007) 1.40
Posttranslational modifications in Cu,Zn-superoxide dismutase and mutations associated with amyotrophic lateral sclerosis. Antioxid Redox Signal (2006) 1.36
Folding of Cu/Zn superoxide dismutase suggests structural hotspots for gain of neurotoxic function in ALS: parallels to precursors in amyloid disease. Proc Natl Acad Sci U S A (2006) 1.31
Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS. Hum Mol Genet (2008) 1.30
SOD1 and amyotrophic lateral sclerosis: mutations and oligomerization. PLoS One (2008) 1.27
Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis. PLoS One (2010) 1.25
Dynamical roles of metal ions and the disulfide bond in Cu, Zn superoxide dismutase folding and aggregation. Proc Natl Acad Sci U S A (2008) 1.23
Rapid profiling of disease alleles using a tunable reporter of protein misfolding. Genetics (2012) 1.20
Copper and zinc metallation status of copper-zinc superoxide dismutase from amyotrophic lateral sclerosis transgenic mice. J Biol Chem (2010) 1.16
An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis. Front Cell Neurosci (2013) 1.15
Mutation-dependent polymorphism of Cu,Zn-superoxide dismutase aggregates in the familial form of amyotrophic lateral sclerosis. J Biol Chem (2010) 1.15
The effects of glutaredoxin and copper activation pathways on the disulfide and stability of Cu,Zn superoxide dismutase. J Biol Chem (2006) 1.15
Superoxide dismutases and superoxide reductases. Chem Rev (2014) 1.12
Loss of metal ions, disulfide reduction and mutations related to familial ALS promote formation of amyloid-like aggregates from superoxide dismutase. PLoS One (2009) 1.11
S-nitrosothiol depletion in amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2006) 1.11
Structural and biophysical properties of metal-free pathogenic SOD1 mutants A4V and G93A. Arch Biochem Biophys (2009) 1.09
Decreased stability and increased formation of soluble aggregates by immature superoxide dismutase do not account for disease severity in ALS. Proc Natl Acad Sci U S A (2011) 1.06
The complex molecular biology of amyotrophic lateral sclerosis (ALS). Prog Mol Biol Transl Sci (2012) 1.05
Regulation of the copper chaperone CCS by XIAP-mediated ubiquitination. Mol Cell Biol (2010) 1.03
Redox regulation of protein misfolding, mitochondrial dysfunction, synaptic damage, and cell death in neurodegenerative diseases. Exp Neurol (2012) 1.02
Metal deficiency increases aberrant hydrophobicity of mutant superoxide dismutases that cause amyotrophic lateral sclerosis. J Biol Chem (2009) 1.02
Strategies for stabilizing superoxide dismutase (SOD1), the protein destabilized in the most common form of familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2010) 1.01
Disulfide-reduced ALS variants of Cu, Zn superoxide dismutase exhibit increased populations of unfolded species. J Mol Biol (2010) 1.01
S-nitrosylation of critical protein thiols mediates protein misfolding and mitochondrial dysfunction in neurodegenerative diseases. Antioxid Redox Signal (2011) 1.00
SOD1 mutations targeting surface hydrogen bonds promote amyotrophic lateral sclerosis without reducing apo-state stability. J Biol Chem (2010) 0.96
Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity. Hum Mol Genet (2010) 0.96
Disulfide scrambling describes the oligomer formation of superoxide dismutase (SOD1) proteins in the familial form of amyotrophic lateral sclerosis. J Biol Chem (2012) 0.94
Thermal fluctuations of immature SOD1 lead to separate folding and misfolding pathways. Elife (2015) 0.94
Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo. Neurobiol Dis (2009) 0.91
SOD1 exhibits allosteric frustration to facilitate metal binding affinity. Proc Natl Acad Sci U S A (2013) 0.91
Nonamyloid aggregates arising from mature copper/zinc superoxide dismutases resemble those observed in amyotrophic lateral sclerosis. J Biol Chem (2010) 0.89
Mechanisms of mutant SOD1 induced mitochondrial toxicity in amyotrophic lateral sclerosis. Front Cell Neurosci (2014) 0.89
Arresting amyloid with coulomb's law: acetylation of ALS-linked SOD1 by aspirin impedes aggregation. Biophys J (2015) 0.88
Redox properties of the disulfide bond of human Cu,Zn superoxide dismutase and the effects of human glutaredoxin 1. Biochem J (2012) 0.85
Non-native soluble oligomers of Cu/Zn superoxide dismutase (SOD1) contain a conformational epitope linked to cytotoxicity in amyotrophic lateral sclerosis (ALS). Biochemistry (2014) 0.85
Pathological roles of wild-type cu, zn-superoxide dismutase in amyotrophic lateral sclerosis. Neurol Res Int (2012) 0.85
In-cell NMR in E. coli to monitor maturation steps of hSOD1. PLoS One (2011) 0.85
Cytosolic superoxide dismutase (SOD1) is critical for tolerating the oxidative stress of zinc deficiency in yeast. PLoS One (2009) 0.84
Palmitoylation of superoxide dismutase 1 (SOD1) is increased for familial amyotrophic lateral sclerosis-linked SOD1 mutants. J Biol Chem (2013) 0.84
The Disulfide Bond, but Not Zinc or Dimerization, Controls Initiation and Seeded Growth in Amyotrophic Lateral Sclerosis-linked Cu,Zn Superoxide Dismutase (SOD1) Fibrillation. J Biol Chem (2015) 0.82
Disulfide bonding in neurodegenerative misfolding diseases. Int J Cell Biol (2013) 0.81
Conformational analysis of misfolded protein aggregation by FRET and live-cell imaging techniques. Int J Mol Sci (2015) 0.80
A seeded propagation of Cu, Zn-superoxide dismutase aggregates in amyotrophic lateral sclerosis. Front Cell Neurosci (2014) 0.80
Conformational Disorder of the Most Immature Cu, Zn-Superoxide Dismutase Leading to Amyotrophic Lateral Sclerosis. J Biol Chem (2015) 0.80
Molecular cloning, characterization and predicted structure of a putative copper-zinc SOD from the camel, Camelus dromedarius. Int J Mol Sci (2012) 0.79
Parkin Protects Against Misfolded SOD1 Toxicity by Promoting Its Aggresome Formation and Autophagic Clearance. Mol Neurobiol (2015) 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
Copper Homeostasis as a Therapeutic Target in Amyotrophic Lateral Sclerosis with SOD1 Mutations. Int J Mol Sci (2016) 0.77
Distinct oxidative cleavage and modification of bovine [Cu- Zn]-SOD by an ascorbic acid/Cu(II) system: Identification of novel copper binding site on SOD molecule. Free Radic Biol Med (2016) 0.77
Screening of Drugs Inhibiting In vitro Oligomerization of Cu/Zn-Superoxide Dismutase with a Mutation Causing Amyotrophic Lateral Sclerosis. Front Mol Biosci (2016) 0.77
Disulfide scrambling in superoxide dismutase 1 reduces its cytotoxic effect in cultured cells and promotes protein aggregation. PLoS One (2013) 0.77
Insights into the role of the unusual disulfide bond in copper-zinc superoxide dismutase. J Biol Chem (2014) 0.76
Enthalpic barriers dominate the folding and unfolding of the human Cu, Zn superoxide dismutase monomer. J Mol Biol (2012) 0.76
A faulty interaction between SOD1 and hCCS in neurodegenerative disease. Sci Rep (2016) 0.76
Small molecules present in the cerebrospinal fluid metabolome influence superoxide dismutase 1 aggregation. Int J Mol Sci (2013) 0.76
Artifacts to avoid while taking advantage of top-down mass spectrometry based detection of protein S-thiolation. Proteomics (2014) 0.76
Probing the free energy landscapes of ALS disease mutants of SOD1 by NMR spectroscopy. Proc Natl Acad Sci U S A (2016) 0.76
Immunochemical characterization on pathological oligomers of mutant Cu/Zn-superoxide dismutase in amyotrophic lateral sclerosis. Mol Neurodegener (2017) 0.75
DNA-triggered aggregation of copper, zinc superoxide dismutase in the presence of ascorbate. PLoS One (2010) 0.75
A misfolded dimer of Cu/Zn-superoxide dismutase leading to pathological oligomerization in amyotrophic lateral sclerosis. Protein Sci (2016) 0.75
S-acylation of SOD1, CCS, and a stable SOD1-CCS heterodimer in human spinal cords from ALS and non-ALS subjects. Sci Rep (2017) 0.75
Susceptibility of Mutant SOD1 to Form a Destabilized Monomer Predicts Cellular Aggregation and Toxicity but Not In vitro Aggregation Propensity. Front Neurosci (2016) 0.75
Identification of Chinese Herbs Using a Sequencing-Free Nanostructured Electrochemical DNA Biosensor. Sensors (Basel) (2015) 0.75
Incorporation of a Reporter Peptide in FPOP Compensates for Adventitious Scavengers and Permits Time-Dependent Measurements. J Am Soc Mass Spectrom (2016) 0.75
Bee venom effects on ubiquitin proteasome system in hSOD1(G85R)-expressing NSC34 motor neuron cells. BMC Complement Altern Med (2013) 0.75
Superoxide dismutase 1 is positively selected to minimize protein aggregation in great apes. Cell Mol Life Sci (2017) 0.75
Transition metal speciation in the cell: insights from the chemistry of metal ion receptors. Science (2003) 5.12
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
Molecular basis of metal-ion selectivity and zeptomolar sensitivity by CueR. Science (2003) 3.08
Oxygen-induced maturation of SOD1: a key role for disulfide formation by the copper chaperone CCS. EMBO J (2004) 2.55
Activation of superoxide dismutases: putting the metal to the pedal. Biochim Biophys Acta (2006) 2.33
Cu(I) recognition via cation-pi and methionine interactions in CusF. Nat Chem Biol (2007) 2.28
Disulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model mice. Proc Natl Acad Sci U S A (2006) 2.22
The unusually stable quaternary structure of human Cu,Zn-superoxide dismutase 1 is controlled by both metal occupancy and disulfide status. J Biol Chem (2004) 1.97
Factors controlling the uptake of yeast copper/zinc superoxide dismutase into mitochondria. J Biol Chem (2003) 1.83
Biological evaluation of pH-responsive polymer-caged nanobins for breast cancer therapy. ACS Nano (2010) 1.82
Metallochaperones and metal-transporting ATPases: a comparative analysis of sequences and structures. Genome Res (2002) 1.80
Zinc availability regulates exit from meiosis in maturing mammalian oocytes. Nat Chem Biol (2010) 1.76
Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis. J Biol Chem (2008) 1.69
Emission ratiometric imaging of intracellular zinc: design of a benzoxazole fluorescent sensor and its application in two-photon microscopy. J Am Chem Soc (2004) 1.66
Fluxes in "free" and total zinc are essential for progression of intraerythrocytic stages of Plasmodium falciparum. Chem Biol (2012) 1.59
A place for thioether chemistry in cellular copper ion recognition and trafficking. Nat Chem Biol (2008) 1.46
Oxygen and the copper chaperone CCS regulate posttranslational activation of Cu,Zn superoxide dismutase. Proc Natl Acad Sci U S A (2004) 1.45
Zinc sparks are triggered by fertilization and facilitate cell cycle resumption in mammalian eggs. ACS Chem Biol (2011) 1.37
Posttranslational modifications in Cu,Zn-superoxide dismutase and mutations associated with amyotrophic lateral sclerosis. Antioxid Redox Signal (2006) 1.36
A physical sciences network characterization of non-tumorigenic and metastatic cells. Sci Rep (2013) 1.33
Folate-mediated intracellular drug delivery increases the anticancer efficacy of nanoparticulate formulation of arsenic trioxide. Mol Cancer Ther (2009) 1.27
Lipid encapsulation of arsenic trioxide attenuates cytotoxicity and allows for controlled anticancer drug release. J Am Chem Soc (2006) 1.27
Tetrathiomolybdate inhibits copper trafficking proteins through metal cluster formation. Science (2009) 1.21
"Clickable" polymer-caged nanobins as a modular drug delivery platform. J Am Chem Soc (2009) 1.18
Polymer-caged lipsomes: a pH-responsive delivery system with high stability. J Am Chem Soc (2007) 1.16
A novel nanoparticulate formulation of arsenic trioxide with enhanced therapeutic efficacy in a murine model of breast cancer. Clin Cancer Res (2010) 1.12
The PcoC copper resistance protein coordinates Cu(I) via novel S-methionine interactions. J Am Chem Soc (2003) 1.12
Zinc requirement during meiosis I-meiosis II transition in mouse oocytes is independent of the MOS-MAPK pathway. Biol Reprod (2010) 1.10
Development of novel therapeutics targeting the urokinase plasminogen activator receptor (uPAR) and their translation toward the clinic. Curr Pharm Des (2011) 1.05
Coencapsulation of arsenic- and platinum-based drugs for targeted cancer treatment. Angew Chem Int Ed Engl (2009) 1.04
A zinc-dependent mechanism regulates meiotic progression in mammalian oocytes. Biol Reprod (2012) 1.04
Spectroscopy of Cu(II)-PcoC and the multicopper oxidase function of PcoA, two essential components of Escherichia coli pco copper resistance operon. Biochemistry (2002) 1.02
Modular polymer-caged nanobins as a theranostic platform with enhanced magnetic resonance relaxivity and pH-responsive drug release. Angew Chem Int Ed Engl (2010) 0.98
An atypical linear Cu(I)-S2 center constitutes the high-affinity metal-sensing site in the CueR metalloregulatory protein. J Am Chem Soc (2003) 0.98
A new zinc-protein coordination site in intracellular metal trafficking: solution structure of the Apo and Zn(II) forms of ZntA(46-118). J Mol Biol (2002) 0.98
Role of CTR4 in the Virulence of Cryptococcus neoformans. MBio (2012) 0.96
Crystal structure and dimerization equilibria of PcoC, a methionine-rich copper resistance protein from Escherichia coli. J Biol Inorg Chem (2002) 0.95
Polymer-caged nanobins for synergistic cisplatin-doxorubicin combination chemotherapy. J Am Chem Soc (2010) 0.94
Acute cytokine-mediated downregulation of the zinc transporter ZnT8 alters pancreatic beta-cell function. J Endocrinol (2010) 0.90
Zinc maintains prophase I arrest in mouse oocytes through regulation of the MOS-MAPK pathway. Biol Reprod (2012) 0.89
Size control of arsenic trioxide nanocrystals grown in nanowells. J Am Chem Soc (2009) 0.88
Nano-encapsulation of arsenic trioxide enhances efficacy against murine lymphoma model while minimizing its impact on ovarian reserve in vitro and in vivo. PLoS One (2013) 0.88
Urokinase plasminogen activator system-targeted delivery of nanobins as a novel ovarian cancer therapy. Mol Cancer Ther (2013) 0.86
Accumulation of cadmium in insulin-producing β cells. Islets (2012) 0.85
Zinc, insulin, and the liver: a ménage à trois. J Clin Invest (2013) 0.82
Anticancer activity of small-molecule and nanoparticulate arsenic(III) complexes. Inorg Chem (2013) 0.82
A copper hyperaccumulation phenotype correlates with pathogenesis in Cryptococcus neoformans. Metallomics (2013) 0.79
Improved anti-proliferative effect of doxorubicin-containing polymer nanoparticles upon surface modification with cationic groups. J Mater Chem (2012) 0.79
Robust structure and reactivity of aqueous arsenous acid-platinum(II) anticancer complexes. Angew Chem Int Ed Engl (2013) 0.78
Design, Implementation, Simulation, and Visualization of a Highly Efficient RIM Microfluidic Mixer for Rapid Freeze-Quench of Biological Samples. Appl Magn Reson (2011) 0.78
Triggered release of pharmacophores from [Ni(HAsO₃)]-loaded polymer-caged nanobin enhances pro-apoptotic activity: a combined experimental and theoretical study. ACS Nano (2011) 0.78
Development and modeling of arsenic-trioxide-loaded thermosensitive liposomes for anticancer drug delivery. J Liposome Res (2010) 0.75