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 cardial expression of free radical scavenger superoxide dismutase 1 and aldehyde dehydrogenase 2 was

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

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

13 two copper-containing mitochondrial enzymes, superoxide dismutase 1 and cytochrome c oxidase, regulat

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

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

16 ed with mitochondrial dysfunction, disturbed superoxide dismutase 1 and Keap1/Nrf2 antioxidant respon

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

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

19 Levels of cyclooxygenase-2 (COX-2), superoxide dismutase-1, and reactive oxygen species (ROS

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

35 y transgenic (Tg) mice expressing the mutant superoxide dismutase-1 G93A.

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

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

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

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

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

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

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

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

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

45 hase II enzymes (heme oxygenase-1, catalase, superoxide dismutase-1) in a time-dependent manner.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

64 Superoxide dismutase-1 mutations decrease protein stabil

65 (ALS), in which astrocytes expressing mutant superoxide dismutase-1 (mutSOD1) kill wild-type motor ne

66 ROS quenching by overexpression of superoxide dismutase 1, or by knockdown of Duox, abolish

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

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

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

70 -1 (CCS), and gene silencing of CCS, but not superoxide dismutase-1, prevents IGF-1- or Cu-induced HI

71 3/STAT3) protein, and (5) coronary arteriole superoxide dismutase 1 protein; or had increases in (6)

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

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

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

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

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

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

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

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

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

81 L-6, IL-17, interferon-gamma (IFN-gamma) and superoxide dismutase 1 (SOD) (P < 0.05).

82 B, IL-6, IL-17, interferon-gamma (IFN-y) and superoxide dismutase 1 (SOD) (P < 0.05).

83 II) coordination sphere at the core of human superoxide dismutase 1 (SOD) with 0.7 pm precision.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

105 ta, mitochondrial dysfunction, and disturbed superoxide dismutase 1 (SOD1) and Keap1/Nrf2 antioxidant

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

107 yotrophic lateral sclerosis (ALS), including SuperOxide Dismutase 1 (SOD1) and Valosin-Containing Pro

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

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

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

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

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

113 Mutations in the gene encoding superoxide dismutase 1 (SOD1) are the second most common

114 Non-natively folded variants of superoxide dismutase 1 (SOD1) are thought to contribute

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

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

117 Mutations in superoxide dismutase 1 (SOD1) cause 15-20% of familial a

118 Mutations in the gene encoding Cu-Zn superoxide dismutase 1 (SOD1) cause a subset of familial

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

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

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

122 Mutations in Cu-Zn superoxide dismutase 1 (SOD1) cause familial forms of am

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

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

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

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

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

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

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

130 Pathogenic variants in the superoxide dismutase 1 (SOD1) gene were the first identi

131 Mutations in the superoxide dismutase 1 (SOD1) gene, resulting in misfold

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

133 and ALS patients harboring mutations in the superoxide dismutase 1 (SOD1) gene.

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

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

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

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

138 ssembly and toxicity of wild-type and mutant superoxide dismutase 1 (SOD1) has been widely examined i

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

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

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

142 throughout the sequence of the gene encoding superoxide dismutase 1 (SOD1) have been linked to toxic

143 the activity and release of a model enzyme, superoxide dismutase 1 (SOD1) immobilized by polyion cou

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

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

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

147 genic proteins amyloid-beta (Abeta), tau and superoxide dismutase 1 (SOD1) in the cerebrospinal fluid

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

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

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

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

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

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

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

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

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

157 cid to lysine (E40K) residue substitution in superoxide dismutase 1 (SOD1) is associated with canine

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

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

160 Superoxide dismutase 1 (SOD1) is the principal cytoplasm

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

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

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

164 onucleotide that mediates the degradation of superoxide dismutase 1 (SOD1) messenger RNA to reduce SO

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

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

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

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

169 yotrophic lateral sclerosis (ALS)-associated superoxide dismutase 1 (SOD1) mutant protein induces cha

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

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

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

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

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

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

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

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

178 Here, we demonstrate that superoxide dismutase 1 (SOD1) plays a crucial role in RO

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

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

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

182 Here, we investigate the association of superoxide dismutase 1 (SOD1) proteins with different fo

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

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

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

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

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

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

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

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

191 Here, we examined potential inhibitors of superoxide dismutase 1 (SOD1) using ThT-fluorescence inc

192 sis (ALS) and mutations in the gene encoding superoxide dismutase 1 (SOD1) were treated with a single

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

194 In superoxide dismutase 1 (SOD1)(G93A) mice, blocking RIPK1

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

196 trophic lateral sclerosis (ALS) mouse model, superoxide dismutase 1 (SOD1)(G93A), revealed that these

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

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

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

200 Here, we show that one such case-Superoxide dismutase 1 (Sod1), a protein that commonly a

201 ng an RfxCas13d variant programmed to target superoxide dismutase 1 (SOD1), a protein whose mutation

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

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

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

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

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

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

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

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

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

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

212 eins, including mutant huntingtin (Htt-Q74), superoxide dismutase 1 (SOD1), tau, and TAR DNA-binding

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

214 sease variants of the ALS-associated protein superoxide dismutase 1 (SOD1), using a biological proteo

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

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

217 he interactions between the immature form of superoxide dismutase 1 (SOD1), which can mislocalize and

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

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

220 form transiently during aggregation of human superoxide dismutase 1 (SOD1), which is known to form mi

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

222 lateral sclerosis-associated protein variant superoxide dismutase 1 (SOD1)-A4V, whereas HSPA1L enhanc

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

224 (ALS), PICs have been previously studied in superoxide dismutase 1 (SOD1)-G93A mice (the standard an

225 sion construct-artificial microRNA targeting superoxide dismutase 1 (SOD1)-in non-human primates (NHP

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

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

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

229 nts for TAR DNA-binding protein (TDP-43) and superoxide dismutase 1 (SOD1).

230 al-sequence-lacking Acb1 and the antioxidant superoxide dismutase 1 (SOD1).

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

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

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

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

235 mportant subtypes are caused by variation in superoxide dismutase 1 (SOD1).

236 roteins, as demonstrated with proinsulin and superoxide dismutase 1 (SOD1).

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

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

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

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

241 ith sporadic ALS and an ALS mouse model with superoxide dismutase 1 (SOD1)/G93A transgenes using nerv

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

243 rotein expression [hemeoxygenase 1 (HO1) and superoxide dismutase 1 (SOD1)].

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

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

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

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

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

249 tic-lethal dependencies of PPM1D, uncovering superoxide dismutase-1 (SOD1) as a potential target for

250 t amplified the negative charge of misfolded superoxide dismutase-1 (SOD1) by acetylating lysine-NH(3

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

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

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

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

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

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

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

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

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

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

261 Neuronal superoxide dismutase-1 (SOD1) inclusion bodies are chara

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

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

264 Superoxide dismutase-1 (SOD1) maturation comprises a str

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

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

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

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

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

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

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

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

273 cells in mice expressing the ALS-associated superoxide dismutase-1 (SOD1)(G93A) mutant decreased spi

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

294 aptotagmin-11, mutant huntingtin, and mutant superoxide dismutase 1, undergo palmitoylation, and rece

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

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

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

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

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

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