Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Here we used an in vitro neuroblastoma cell line transfection system to monitor wild-type and mutant forms of SOD1 fusion proteins containing either a Cherry or an enhanced green fluorescent protein (EGFP) tag.
|
31336405 |
2019 |
Childhood Neuroblastoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Immunofluorescence and co-immunoprecipitation experiments showed that the 14-3-3ɛ and θ isoforms interact with mutant SOD1 aggregates in the juxtanuclear quality control compartment of N2a neuroblastoma cells.
|
28666328 |
2017 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We observed an increased total iron content in G93A-SOD1 SH-SY5Y neuroblastoma cells compared to wild-type (WT)-SOD1 cells. mRNA expression for transferrin receptor 1 (TfR1) and divalent metal transporter 1 was increased in G93A-SOD1 cells, which was in accordance with higher iron uptake.
|
23178912 |
2013 |
Childhood Neuroblastoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
SOD1 misfolding and oxidation was also detected using immunocytochemistry and quantitative immunoprecipitation of human neuroblastoma SH-SY5Y cells as well as cultured murine spinal neural cells transgenic for human wtSOD1, which were transiently transfected with human cytosolic mutant FUS or TDP43, or wtTDP43.
|
22493728 |
2012 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Overexpression of a familial mutant form of superoxide dismutase 1 (SOD1) (G93A) in neuroblastoma cells resulted in a similar reduction of IGF-1Rβ protein.
|
22875931 |
2012 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
To characterize the cellular response to mitochondrial perturbations at the level of gene expression and alternative pre-mRNA splicing we used splicing-sensitive microarrays to profile human neuroblastoma SH-SY5Y cells treated with paraquat, a neurotoxic herbicide that induces the formation of reactive oxygen species and causes mitochondrial damage in animal models, and SH-SY5Y cells stably expressing the mutant G93A-SOD1 protein, one of the genetic causes of ALS.
|
21120952 |
2011 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We also detected oligomer formation of L84V SOD1 in L84V SOD1-expressing human neuroblastoma cells.
|
20816908 |
2010 |
Childhood Neuroblastoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Therefore, the aim of the present study was to examine in the human neuroblastoma SH-SY5Y cell line the impact of a typical (haloperidol) and an atypical (quetiapine) antipsychotic on the expression of genes encoding the key enzymes of the antioxidant metabolism (Cu, Zn superoxide dismutase; Mn superoxide dismutase; glutathione peroxidase; catalase) and enzymes of the glutathione metabolism (gamma-glutamyl cysteine synthetase, glutathione-S-transferase, gamma-glutamyltranspeptidase, glutathione reductase).
|
19101687 |
2009 |
Childhood Neuroblastoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We demonstrated that SOD1 depletion induced, only in neuroblastoma cells, a decrease in actin and beta-tubulin content and accumulation of neurofilament light chain and Tau proteins.
|
18239850 |
2008 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We detected this interaction both in spinal cord extracts of mutant SOD1(G93A) transgenic mice and in cultured neuroblastoma cells.
|
17403032 |
2007 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
SH-SY5Y neuroblastoma cells transfected with the G93A mutant of SOD1 typical for familial ALS (G93A-SOD1) were more vulnerable to the neurotoxic action of pneumolysin and to the attack of monocytes stimulated by Pam3CSK4 than SH-SY5Y cells transfected with wild-type human SOD1.
|
17997855 |
2007 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Previously, we reported that overexpression of the mitochondrial antioxidant manganese superoxide dismutase (MnSOD or SOD2) attenuates cytotoxicity induced by expression of the G37R-SOD1 mutant in a human neuroblastoma cell culture model of ALS.
|
17394531 |
2007 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In the light of the possibility that different SOD1 entities could be expressed also in other neurodegenerative disorders, as a sort of unifying event with AD and PD, we have investigated amyotrophic lateral sclerosis (ALS) using human neuroblastoma SH-SY5Y cells with mutated SOD1 gene H46R as cellular model.
|
17987632 |
2007 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
To understand better the role of these mutations in the pathophysiology of FALS we have compared the pattern of proteins expressed in human neuroblastoma SH-SY5Y cell line with those of cell lines transfected with plasmids expressing the wild-type human SOD1 and the H46R and G93A mutants.
|
17979159 |
2007 |
Childhood Neuroblastoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
In order to investigate the role of Hsp27 in mutant SOD1-dependent cell death, we used mutant and wild type SOD1 overexpressing N2a mouse neuroblastoma cells.
|
16806187 |
2006 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In order to investigate the basis of the tissue specificity of mutant SOD1 we compared the effect of the continuous expression of wild-type or mutant (G93A) human SOD1 on mitochondrial morphology in the NSC-34 motoneuronal-like, the N18TG2 neuroblastoma and the non-neuronal Madin-Darby Canine Kidney (MDCK) cell lines.
|
16903849 |
2006 |
Childhood Neuroblastoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
Overexpression of mutated Cu,Zn-SOD in neuroblastoma cells results in cytoskeletal change.
|
15456693 |
2005 |
Childhood Neuroblastoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Impairment of glutamate transport and increased vulnerability to oxidative stress in neuroblastoma SH-SY5Y cells expressing a Cu,Zn superoxide dismutase typical of familial amyotrophic lateral sclerosis.
|
15670639 |
2005 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Motoneuron-neuroblastoma hybrid (VSC 4.1) cells expressing mutant SOD1, when treated with copper chloride, showed reduced viability and increased levels of endogenous peroxides.
|
15812313 |
2005 |
Childhood Neuroblastoma
|
0.100 |
Biomarker
|
disease |
BEFREE |
We have investigated the functional effects of RNAi-mediated silencing of mutant SOD1 in cultured murine neuroblastoma cells.
|
14981234 |
2004 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We show that human glioblastoma cells expressing G93A-SOD1 induce activation of caspase-1, release of cytokines, and activation of apoptotic pathways in cocultured human neuroblastoma cells also expressing G93A-SOD1.
|
15208263 |
2004 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We developed an in vitro disease model with motor neuron-neuroblastoma hybrid cells (VSC4.1) constitutively expressing a mutant (G93A) SOD1.
|
15289674 |
2004 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We report that the expression of mutant G93A copper/zinc superoxide dismutase (SOD1), associated with familial amyotrophic lateral sclerosis, specifically causes a decrease in MTT reduction rate and ATP levels and an increase in both cytosolic and mitochondrial reactive oxygen species (ROS) production in human neuroblastoma SH-SY5Y cells compared to cells overexpressing wild-type SOD1 and untransfected cells.
|
12901835 |
2003 |
Childhood Neuroblastoma
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Overexpression of Cu-Zn superoxide dismutase protects neuroblastoma cells against dopamine cytotoxicity accompanied by increase in their glutathione level.
|
12941444 |
2003 |
Childhood Neuroblastoma
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We demonstrated that expression of the fully active G93A Cu,Zn superoxide dismutase mutant in neuroblastoma cells is associated with an increased level of oxidatively modified proteins, in terms of carbonylated residues.
|
12753090 |
2003 |