ライフサイエンスコーパス: superoxide dismutase 1

1 hy was prevented by transgenic expression of superoxide dismutase 1.

2 axin-3 as well as mutant alpha-synuclein and superoxide dismutase 1.

3 vels of superoxide in a yeast mutant lacking superoxide dismutase 1.

4 which accumulate intracellular aggregates of superoxide dismutase-1.

5 endothelial nitric oxide synthase (39%), and superoxide dismutase-1 (45%).

6 events palmitoylation, leads to reduction in superoxide dismutase-1 activity in vivo and in vitro, an

7 Both were different from superoxide dismutase-1 aggregates generated in vitro und

8 ng the novel palmitoylproteins identified is superoxide dismutase-1, an intensively studied enzyme th

9 Quercetin increased the expression of superoxide dismutase 1 and 2, and reduced the levels of

10 Downstream of Nrf2, levels of oPMN superoxide dismutase 1 and catalase were decreased in se

11 th downregulation of the antioxidant enzymes superoxide dismutase 1 and catalase, and activation of t

12 anscription and translation of antioxidants, superoxide dismutase 1 and glutathione peroxidase-1, wer

13 o oxidized glutathione, and up-regulation of superoxide dismutase 1 and heat shock protein 70, all co

14 es, reactive oxidative species scavenging by superoxide dismutase-1 and superoxide dismutase-2.

15 antioxidant enzymes, including catalase and superoxide dismutases 1 and 2.

16 tholog of human DJ-1/Park7, gamma-synuclein, superoxide dismutase 1), anti-oxidant proteins (peroxire

17 uronal cytoplasmic inclusions that bind anti-superoxide dismutase 1 antibodies.

18 ies showed that astrocytes expressing mutant superoxide dismutase 1 are toxic to normal motoneurons.

19 We find both compounds interact with superoxide dismutase-1 at a key region identified at the

20 ain increased vulnerability, including SOD1 (superoxide dismutase 1), catalase, glutathione S-transfe

21 Mutations in the gene encoding Cu/Zn superoxide dismutase-1 cause amyotrophic lateral scleros

22 terfering RNA targeting copper chaperone for superoxide dismutase 1 (CCS) suppressed hypoxia-induced

23 coimmunoprecipitates with a Cu chaperone for superoxide dismutase-1 (CCS), and gene silencing of CCS,

24 A subset of superoxide dismutase 1 (Cu/Zn-SOD1) mutants that cause f

25 ds previously were reported to inhibit Cu/Zn superoxide dismutase 1 dependent protein aggregation and

26 Intervention to stabilize the superoxide dismutase-1 dimer and inhibit aggregation is

27 o be an intermediate in the pathway from the superoxide dismutase-1 dimer to aggregate.

28 t a key region identified at the core of the superoxide dismutase-1 fibrillar aggregates, beta-barrel

29 from the expression of mutant proteins (G85R superoxide dismutase 1; G59S p150(glued)) that cause fam

30 Mice with high numbers of the Cu/Zn superoxide dismutase-1 G93A transgene (SOD1(G93A) G1H) h

31 express the G93A mutation of the human Cu-Zn superoxide dismutase 1 gene (hSOD1(G93A) mice) are a com

32 that develop DM are homozygous for a common superoxide dismutase 1 gene (SOD1) mutation.

33 er-expressing the G93A mutation of the Cu/Zn superoxide dismutase 1 gene) of ALS enters a progressive

34 Expression of superoxide dismutase 1, glutathione S-transferase-pi, an

35 made of tau, alpha-synuclein, huntingtin and superoxide dismutase 1 has been demonstrated, revealing

36 ve post-translational modifications of human superoxide dismutase 1 (hSOD1) in the amyotrophic latera

37 uced accumulation of chloroplast copper/zinc superoxide dismutase 1 (HvSOD1), whereas loss of functio

38 uced accumulation of chloroplast copper/zinc superoxide dismutase 1 (HvSOD1), whereas loss of functio

39 ly, when overproduced, Atx1p substitutes for superoxide dismutase 1 in preventing oxidative damage; h

40 ns (polyglutamine, huntingtin, ataxin-1, and superoxide dismutase-1) inhibits clathrin-mediated endoc

41 Mutation in superoxide dismutase-1 is a cause of the fatal paralytic

42 bdb) model of diabetic polyneuropathy and 2) superoxide dismutase 1 knockout (Sod1(-/-) ) mouse model

43 d significantly higher glutathione and Cu-Zn superoxide dismutase 1 levels compared with ALS/Lt islet

44 ssion of the G985R and G93A mutated forms of superoxide dismutase 1 (linked to familial amyotrophic l

45 specifically discussed: transthyretin, p53, superoxide dismutase 1, lysozyme, serum amyloid A, prion

46 the transcriptional profile of human mutant superoxide dismutase 1 motor neurons has remained undisc

47 se, delays disease progression in the mutant superoxide dismutase 1 mouse model of amyotrophic latera

48 Similarly, two mutant copper zinc superoxide dismutase 1 (mSOD1) mouse models of familial

49 tion and disease propagation in mutant human superoxide dismutase 1 (mSOD1)-mediated amyotrophic late

50 s of reconstructed wild-type (WT) and mutant superoxide dismutase-1 (mSOD1) motoneurons.

51 The mechanisms of human mutant superoxide dismutase-1 (mSOD1) toxicity to motor neurons

52 transgenic (tg) mice harboring human mutant superoxide dismutase-1 (mSOD1), a model of ALS.

53 motor neuron degeneration produced by mutant superoxide dismutase-1 (mSOD1), the only proven cause of

54 Recent work, using mutant superoxide dismutase 1 murine models and in vitro cultur

55 identified F- and S-type motoneurons in the superoxide dismutase-1 mutant G93A (mSOD1), a mouse mode

56 Mice that overexpress the human Cu,Zn superoxide dismutase-1 mutant G93A develop a delayed and

57 sing an amyotrophic lateral sclerosis-linked superoxide dismutase-1 mutation led to the idea that pro

58 Superoxide dismutase-1 mutations decrease protein stabil

59 Studies in mutant superoxide dismutase 1 over-expressing amyotrophic later

60 rimary astrocytes isolated from mutant human superoxide dismutase 1-overexpressing mice as well as hu

61 on, thereby supporting a functional role for superoxide dismutase-1 palmitoylation.

62 -1 (CCS), and gene silencing of CCS, but not superoxide dismutase-1, prevents IGF-1- or Cu-induced HI

63 3/STAT3) protein, and (5) coronary arteriole superoxide dismutase 1 protein; or had increases in (6)

64 missense mutations, such as by mutations in superoxide dismutase-1 protein.

65 rone activity toward its physiologic target, superoxide dismutase 1, rather than proteasomal degradat

66 g to laser captured motor neurons from human superoxide dismutase 1-related amyotrophic lateral scler

67 ellular and animal model work has focused on superoxide dismutase 1-related amyotrophic lateral scler

68 ssed genes in spinal cord motor neurons from superoxide dismutase 1-related amyotrophic lateral scler

69 e in the propagation of motoneuron injury in superoxide dismutase 1-related amyotrophic lateral scler

70 ion of in vivo and in vitro model systems of superoxide dismutase 1-related amyotrophic lateral scler

71 termine those pathways dysregulated in human superoxide dismutase 1-related neurodegeneration and to

72 ymptomatic transgenic mice expressing mutant superoxide dismutase 1 revealed two striking changes.

73 Mutations in the copper/zinc superoxide dismutase 1 (SOD-1) gene have previously been

74 were down-regulated: copper-zinc-containing superoxide dismutase 1 (SOD-1), insulin-like growth fact

75 neration: In mice that overexpress wild-type superoxide dismutase-1 (SOD(WT)), axons show chronic tra

76 Mutations in human Cu/Zn superoxide dismutase-1 (SOD) cause approximately 20% of

77 ral sclerosis (FALS)-associated mutant Cu/Zn superoxide dismutase-1 (SOD) induces apoptosis of neuron

78 One, caused by a G86R mutation in the superoxide dismutase-1 (SOD-1) gene associated with fami

79 om an in silico screen that stabilize mutant superoxide dismutase-1 (SOD-1) linked to familial amyotr

80 e effects of overexpression of Cu(2+)/Zn(2+) superoxide dismutase-1 (SOD-1) on indexes of renal injur

81 Here, we show superoxide dismutase-1 (SOD-1), an enzyme that converts

82 duced expression of the antioxidant enzymes: superoxide-dismutase 1 (SOD-1), SOD-2, and catalase thro

83 e enhanced by expression of a mutant form of superoxide dismutase 1 (SOD1 G93A) that causes astrocyte

84 Mice deficient in superoxide dismutase 1 (Sod1(-/-) mice) have increased o

85 ouse model expressing a mutant form of human superoxide dismutase 1 (SOD1(G93A) ).

86 Mutant superoxide dismutase 1 (SOD1(G93A)) expression in astroc

87 murine model in which a pathogenic mutant of superoxide dismutase 1 (SOD1(G93A)) is expressed in an A

88 We administered fenofibrate to superoxide dismutase 1 (SOD1(G93A)) mice daily prior to

89 Using a mouse model of ALS expressing mutant superoxide dismutase 1 (SOD1(G93A)), we show that motor

90 Overexpression of a familial mutant form of superoxide dismutase 1 (SOD1) (G93A) in neuroblastoma ce

91 Mutations in the gene encoding superoxide dismutase 1 (SOD1) account for about 20% of t

92 ive diseases such as ALS, where mutations of superoxide dismutase 1 (SOD1) account for about 20% of t

93 Mutant superoxide dismutase 1 (SOD1) action within non-neuronal

94 storing the activity of antioxidant enzymes, superoxide dismutase 1 (SOD1) and peroxiredoxin-4 (PRDX4

95 Mutations in superoxide dismutase 1 (SOD1) are associated with famili

96 Changes in the redox state of superoxide dismutase 1 (SOD1) are associated with the on

97 Mutations in the gene superoxide dismutase 1 (SOD1) are causative for familial

98 Mutations in copper/zinc superoxide dismutase 1 (SOD1) are found in approximately

99 Mutations in superoxide dismutase 1 (SOD1) are responsible for a subs

100 and gene expression analysis, we now propose superoxide dismutase 1 (SOD1) as the most likely target

101 Here, we report that inhibition of superoxide dismutase 1 (SOD1) by the small molecule ATN-

102 Mutations in superoxide dismutase 1 (SOD1) cause amyotrophic lateral

103 Mutations in superoxide dismutase 1 (SOD1) cause familial ALS.

104 One of the mechanisms by which mutations in superoxide dismutase 1 (SOD1) cause familial amyotrophic

105 ch DNA-damaging agents, NGF deprivation, and superoxide dismutase 1 (SOD1) depletion evoke apoptosis

106 uired for copper-dependent activation of the superoxide dismutase 1 (SOD1) during spore germination.

107 rom mice carrying ALS-linked mutant forms of superoxide dismutase 1 (SOD1) exhibit an increased sensi

108 domain that requires the host cell cofactor superoxide dismutase 1 (SOD1) for activation, while the

109 pase-11 is upregulated in the spinal cord of superoxide dismutase 1 (SOD1) G93A transgenic mice, a mo

110 Mutations of the superoxide dismutase 1 (SOD1) gene are linked to amyotro

111 clear genetic link to point mutations in the superoxide dismutase 1 (SOD1) gene has been shown in a s

112 ALS based upon dominant mutations within the superoxide dismutase 1 (SOD1) gene, which account for <2

113 ed, of which 20% are due to mutations in the superoxide dismutase 1 (SOD1) gene.

114 Transgenic overexpression of Cu(+2)/Zn(+2) superoxide dismutase 1 (SOD1) harboring an amyotrophic l

115 ced aggregation of the dimeric enzyme Cu, Zn superoxide dismutase 1 (SOD1) has been implicated in the

116 Superoxide dismutase 1 (Sod1) has been known for nearly

117 Transgenic mouse models expressing mutant superoxide dismutase 1 (SOD1) have been critical in furt

118 To date, 146 different mutations in superoxide dismutase 1 (SOD1) have been identified in pa

119 Mutations in the enzyme superoxide dismutase 1 (SOD1) have been linked to the ne

120 shown that ALS-associated mutations in Cu/Zn superoxide dismutase 1 (SOD1) impair axonal transport of

121 e in mice harboring mutations of copper-zinc superoxide dismutase 1 (SOD1) in familial amyotrophic la

122 rew-like structure of a cytotoxic segment of superoxide dismutase 1 (SOD1) in its oligomeric state.

123 al behavior of the pathogenic A4V variant of superoxide dismutase 1 (SOD1) in the metal-free (apo) fo

124 ation kinetics of the ALS-associated protein superoxide dismutase 1 (SOD1) in vitro and in transgenic

125 Insertion of copper into superoxide dismutase 1 (SOD1) in vivo requires the coppe

126 ghput screening assay targeting mutant Cu/Zn superoxide dismutase 1 (SOD1) induced toxicity and aggre

127 cological inhibition or genetic knockdown of superoxide dismutase 1 (SOD1) inhibits the functional ho

128 Mutations in the enzyme superoxide dismutase 1 (SOD1) initiate a progressive mot

129 Superoxide dismutase 1 (SOD1) interacts with the NOX-Rac

130 e find that injection of oligomers of mutant superoxide dismutase 1 (SOD1) into the cytoplasm of inve

131 Superoxide dismutase 1 (SOD1) is an abundant copper/zinc

132 Mutant human Cu/Zn superoxide dismutase 1 (SOD1) is associated with motor n

133 -of-function" toxic property of mutant Cu-Zn superoxide dismutase 1 (SOD1) is involved in the pathoge

134 The Cu- and Zn-containing superoxide dismutase 1 (SOD1) largely obtains Cu in vivo

135 oxidative damage through genetic ablation of superoxide dismutase 1 (SOD1) leads to abnormalities in

136 We show that increased plasma superoxide dismutase 1 (SOD1) levels are statistically s

137 Here, we report that placing mutant superoxide dismutase 1 (SOD1) mice in a leptin-deficient

138 xamined in ALS2-deficient mice and in mutant superoxide dismutase 1 (SOD1) mice that develop ALS-like

139 asked if decreasing metabolism in the mutant superoxide dismutase 1 (SOD1) mouse model of ALS (G93A S

140 Here we show that, in vitro, mutant superoxide dismutase 1 (SOD1) mouse oligodendrocytes ind

141 ally bind and neutralize misfolded and toxic superoxide dismutase 1 (SOD1) mutant proteins may find a

142 Two of the models (polyalanine (37A) and superoxide dismutase 1 (SOD1) mutants A4V and G85R) accu

143 re that amyotrophic lateral sclerosis-linked superoxide dismutase 1 (SOD1) mutants with different bio

144 associated with the most common copper/zinc superoxide dismutase 1 (SOD1) mutation, an alanine for v

145 Superoxide dismutase 1 (SOD1) mutations account for up t

146 owever, the mechanistic relationship between superoxide dismutase 1 (SOD1) mutations and human diseas

147 as abrogated by transgenic overexpression of superoxide dismutase 1 (SOD1) or an SOD1 mimetic.

148 cells, were either decreased by a mimetic of superoxide dismutase 1 (SOD1) or by overexpressing SOD1

149 Mutations in superoxide dismutase 1 (SOD1) polypeptides cause a form

150 ophic lateral sclerosis-associated cytosolic superoxide dismutase 1 (SOD1) protein between motor neur

151 NF-L protein and to coaggregation of mutant superoxide dismutase 1 (SOD1) protein.

152 lateral sclerosis (ALS)-causing mutations in superoxide dismutase 1 (SOD1) provokes noncell autonomou

153 on in a well-validated cell-culture model of superoxide dismutase 1 (SOD1) related familial MND (fMND

154 Determining the composition of aggregated superoxide dismutase 1 (SOD1) species associated with am

155 Although superoxide dismutase 1 (SOD1) stands out as a relatively

156 one or more toxic function(s) on copper/zinc superoxide dismutase 1 (SOD1) that impair motor neuron v

157 ic lateral sclerosis (ALS) have mutations in superoxide dismutase 1 (SOD1) that increase the denitros

158 Notably, G85R is a mutant version of Cu/Zn superoxide dismutase 1 (SOD1) that is unable to reach na

159 from mature motor neurons attenuates mutant superoxide dismutase 1 (SOD1) toxicity.

160 r assembly of cytochrome c oxidase (CcO) and superoxide dismutase 1 (Sod1) within the intermembrane s

161 immunity were investigated in the CNS of the Superoxide Dismutase 1 (SOD1)(G93A) transgenic mouse mod

162 Mutations in copper/zinc superoxide dismutase 1 (SOD1), a genetic cause of human

163 Superoxide dismutase 1 (SOD1), a key antioxidant enzyme

164 Interestingly, the addition of bovine liver superoxide dismutase 1 (SOD1), a known activator of P. a

165 x-deficient mice with mice expressing mutant superoxide dismutase 1 (SOD1), a transgenic model of fam

166 signal sequence lacking cytoplasmic protein, superoxide dismutase 1 (SOD1), and its mutant form linke

167 ALS patients known not to carry mutations in superoxide dismutase 1 (SOD1), as well as from 75 sporad

168 Copper chaperone for superoxide dismutase 1 (SOD1), CCS, is the physiological

169 ies by overexpressing the antioxidant enzyme superoxide dismutase 1 (SOD1), in a transgenic mouse, in

170 human and yeast copper chaperones (CCS) for superoxide dismutase 1 (SOD1), long thought to exclusive

171 ction and determined the in vivo kinetics of superoxide dismutase 1 (SOD1), mutation of which causes

172 we found that antisense oligonucleotides to superoxide dismutase 1 (SOD1), one of the most abundant

173 Mutations in copper/zinc superoxide dismutase 1 (SOD1), primary causes of human a

174 Mutations in superoxide dismutase 1 (SOD1), TAR DNA-binding protein (

175 Mutations in superoxide dismutase 1 (SOD1), the only proven cause of

176 In mice expressing ALS-associated mutant superoxide dismutase 1 (SOD1), VEGF mRNA expression in t

177 uppressed by oligomers of mutant human Cu/Zn superoxide dismutase 1 (SOD1), which are associated with

178 For mutated superoxide dismutase 1 (SOD1), which causes familial amy

179 was enriched in astrocytes expressing mutant superoxide dismutase 1 (SOD1), which causes familial amy

180 he dynamic motions within the beta-barrel of superoxide dismutase 1 (SOD1), which previously were imp

181 viously been found to be inhibitors of Cu/Zn superoxide dismutase 1 (SOD1)-dependent protein aggregat

182 ngly slows disease in mouse models of mutant superoxide dismutase 1 (SOD1)-induced amyotrophic latera

183 Recent studies suggest that superoxide dismutase 1 (SOD1)-linked amyotrophic lateral

184 croglial phenotypes in preclinical stages of superoxide dismutase 1 (SOD1)-mutant-mediated disease.

185 proproteins and within the cytosol to supply superoxide dismutase 1 (Sod1).

186 ALS that is not associated with mutations in superoxide dismutase 1 (SOD1).

187 sease caused by inherited mutations in Cu/Zn superoxide dismutase 1 (SOD1).

188 d with "gain of function" mutations in Cu/Zn superoxide dismutase 1 (SOD1).

189 nly caused by mutations in the gene encoding superoxide dismutase 1 (SOD1).

190 ys in the liver, including downregulation of superoxide dismutase 1 (Sod1).

191 ion of the reactive oxygen species scavenger superoxide dismutase 1 (SOD1).

192 racellular proteins with similarity to Cu/Zn superoxide dismutase 1 (SOD1).

193 S100; transcription factor ERG; antioxidant superoxide dismutase 1 (SOD1); chloride intracellular ch

194 f mice expressing the G93A mutation of human superoxide dismutase 1 (SOD1-G93A).

195 Mutations in superoxide dismutase 1 (SOD1; EC 1.15.1.1) are responsib

196 gene coding for the mutated G93A form of the superoxide dismutase-1 (SOD1(G93A)), even when treatment

197 sregulation has been shown to correlate with superoxide dismutase-1 (SOD1) aggregation in a cellular

198 f ALS cases are sporadic, mutations in Cu-Zn superoxide dismutase-1 (SOD1) are causative for 10-20% o

199 Mutations in superoxide dismutase-1 (SOD1) cause a form of the fatal

200 Mutations in Cu/Zn superoxide dismutase-1 (SOD1) cause familial ALS but the

201 Mutation in superoxide dismutase-1 (SOD1) causes the inherited degen

202 Mice expressing variants of superoxide dismutase-1 (SOD1) encoding C-terminal trunca

203 This article investigates how the rate of superoxide dismutase-1 (SOD1) fibrillization is affected

204 (ALS) cases were linked to mutations in the superoxide dismutase-1 (SOD1) gene, a substantial propor

205 ) cases are associated with mutations of the superoxide dismutase-1 (SOD1) gene, and the 'D90A' mutat

206 forms of ALS are linked to mutations in the superoxide dismutase-1 (SOD1) gene.

207 The acylation of lysine residues in superoxide dismutase-1 (SOD1) has been previously shown

208 increasing evidence that toxicity of mutant superoxide dismutase-1 (SOD1) in amyotrophic lateral scl

209 The copper-zinc superoxide dismutase-1 (SOD1) is a highly structured pro

210 Superoxide dismutase-1 (SOD1) is a ubiquitous, Cu and Zn

211 osis (ALS) cases result from impaired mutant superoxide dismutase-1 (SOD1) maturation.

212 Rodent models in which the superoxide dismutase-1 (SOD1) mutation is overexpressed

213 d in transgenic mice expressing both APP and superoxide dismutase-1 (SOD1) or in APP transgenics in w

214 Abnormal assemblies formed by misfolded superoxide dismutase-1 (SOD1) proteins are the likely ca

215 , we have used mice expressing a mutation in superoxide dismutase-1 (SOD1) that is linked to amyotrop

216 trochemical setup and use the specificity of superoxide dismutase-1 (SOD1) to show, for the first tim

217 Mice expressing human ALS mutant superoxide dismutase-1 (SOD1) were crossed with mice tha

218 ly gene (IEG) proteins and the HSA21 protein superoxide dismutase-1 (SOD1) were found in the hippocam

219 In this study, we show that mutant superoxide dismutase-1 (SOD1), a cause of familial ALS,

220 ALS) is linked to over 90 point mutations in superoxide dismutase-1 (SOD1), a dimeric metalloenzyme.

221 als essential for the activity of the enzyme superoxide dismutase-1 (SOD1), a potential target for an

222 ))ATSM enhanced the association of DJ-1 with superoxide dismutase-1 (SOD1), paralleled by significant

223 use models of familial ALS expressing mutant superoxide dismutase-1 (SOD1), TAR DNA-binding protein 4

224 Mutation in superoxide dismutase-1 (SOD1), which is a cause of ALS,

225 electrophoresis to measure the net charge of superoxide dismutase-1 (SOD1)--whose aggregation causes

226 disease, as has been shown for mutations in superoxide dismutase-1 (SOD1).

227 redox stress caused by dominant mutations in superoxide dismutase-1 (SOD1).

228 lox and by overexpression of wild-type human superoxide dismutase-1 (SOD1).

229 e carry mutations in the gene encoding Cu/Zn superoxide dismutase-1 (SOD1).

230 ch using a standard animal model, the mutant superoxide dismutase-1 (SOD1)mouse, has revealed deficit

231 nherited ALS caused by dominant mutations in superoxide-dismutase 1 (SOD1) were identified by using g

232 c slice cultures from a mutant form of human superoxide dismutase 1 (SOD1G93A) mouse model of ALS all

233 oterenol and 5-fluorouridine, highlighted as superoxide dismutase-1 stabilizers.

234 ed intraplatelet expression of p47(phox) and superoxide dismutase-1, suggesting a mechanistic pathway

235 cluding mutant FUS (Fused in sarcoma), SOD1 (superoxide dismutase 1), TDP43 (TAR DNA-binding protein

236 proteins, such as hemoglobin alpha genes and superoxide dismutase 1, that have network functions asso

237 An archetype example of this is superoxide dismutase-1, the first genetic factor to be l

238 properties change in the presence of mutant superoxide dismutase 1 to contribute to motoneuron injur

239 ouse model expressing a mutant form of human superoxide dismutase-1 with a Gly93-->Ala substitution (

240 ns in mice expressing a mutant form of human superoxide dismutase-1 with a Gly93-->Ala substitution (

241 Additionally, mice transgenic for human superoxide dismutase-1 with G85R mutation show no differ

242 Co-expression of wild-type human superoxide dismutase 1 (WT-hSOD1) with ALS mutant hSOD1

243 cent lumbar MN cell bodies from ChAT-eGFP or superoxide dismutase 1-yellow fluorescent protein (SOD1Y

244 They include 5 glutathione-S-transferases, superoxide dismutase 1, zeta crystallin, a NADPH quinone