ライフサイエンスコーパス: expanded polyglutamine

1 the link between intranuclear expression of expanded polyglutamine and neuronal dysfunction remains

2 r toxicity may be due to interaction between expanded polyglutamine and the histone acetyltransferase

3 s most prominent in yeast expressing nuclear expanded polyglutamine and was similar to profiles of ye

4 elation between the reactivity to 3B5H10, an expanded polyglutamine antibody that recognizes a compac

5 oting pathways, also reduces toxicity of the expanded polyglutamine AR in MN-1 cells, in a manner dep

6 We show that expanded polyglutamine AR is phosphorylated by Akt.

7 study, we use these two forms of GR to study expanded polyglutamine AR protein in different cell cont

8 , transcriptional activation and toxicity of expanded polyglutamine AR.

9 n reduces aggregation and cytotoxicity of an expanded polyglutamine ataxin-3 fragment.

10 nizes a two-stranded hairpin conformation of expanded polyglutamine believed to be associated with a

11 lations, which indicate that constructs with expanded polyglutamine beta-strands are stabilized by ma

12 antibody 1C2 is known preferentially to bind expanded polyglutamine, but we find that it also binds a

13 is a neurodegenerative disorder caused by an expanded polyglutamine (CAG) trinucleotide expansion in

14 In mouse models, proteins with expanded polyglutamine cause transcriptional dysregulati

15 in vitro for a model of disease in which an expanded polyglutamine-containing fragment recruits full

16 TT) within the exon1 region, resulting in an expanded polyglutamine-containing mHTT exon1 protein tha

17 The expanded polyglutamine-containing mutant huntingtin (mHT

18 cal phenotype suggesting that in contrast to expanded polyglutamine-containing proteins, neither the

19 association between aberrant accumulation of expanded polyglutamine-dependent insoluble protein speci

20 evels of CBP are reduced in cells expressing expanded polyglutamine despite increased levels of CBP m

21 gate formed by the huntingtin exon 1 with an expanded polyglutamine domain (103QP) represents a bona

22 The pathological form of ataxin-3 with an expanded polyglutamine domain also associates with the n

23 oceeds via an intramolecular collapse of the expanded polyglutamine domain and discuss the implicatio

24 An expanded polyglutamine domain in huntingtin underlies th

25 on's disease (HD) gene, is expressed with an expanded polyglutamine domain in the brain and in nonneu

26 omes expanded, resulting in expression of an expanded polyglutamine domain in the disease gene produc

27 nerative disorder initiated by an abnormally expanded polyglutamine domain in the huntingtin protein.

28 Expanded polyglutamine domain proteins possess propertie

29 ated with proteins that contain an unusually expanded polyglutamine domain, the best known of which i

30 Abnormally expanded polyglutamine domains are associated with at le

31 Proteins with expanded polyglutamine domains cause eight inherited neu

32 h short polyglutamine domains, proteins with expanded polyglutamine domains display unique protein in

33 Abnormally expanded polyglutamine domains in proteins are associate

34 Abnormally expanded polyglutamine domains in proteins are associate

35 ive inhibition of pathologic interactions of expanded polyglutamine domains with themselves or other

36 Consistent with an effect on SAGA, nuclear expanded polyglutamine enhanced the toxicity of a deleti

37 In yeast, aggregation and toxicity of the expanded polyglutamine fragment of human huntingtin stri

38 It has been well documented that expanded polyglutamine fragments, cleaved from their res

39 ding sequence of the huntingtin gene, and an expanded polyglutamine (>37Q) tract in the protein.

40 to pathogenesis, with protein context of the expanded polyglutamine having key roles in disease-speci

41 ersions of the mouse Hprt protein containing expanded polyglutamine (HprtQ150).

42 ssing an N-terminal huntingtin fragment with expanded polyglutamine (Htt-N63-148Q).

43 on of the N-terminal fragment containing the expanded polyglutamine (HTTQ94) of mHTT is able to promo

44 m one affected gene (PHO84) was repressed by expanded polyglutamine in a reporter gene assay, and thi

45 expressing a pathogenic human Atxn1 with an expanded polyglutamine in cerebellar Purkinje cells.

46 at-shock factors was caused by expression of expanded polyglutamine in either the nucleus or cytoplas

47 ession of an aggregate prone protein such as expanded polyglutamine in IBMPFD mutant cells results in

48 Because the expression of expanded polyglutamine in selected neuronal populations

49 Expanded polyglutamine induced a marked increase in expr

50 lement-binding protein (CREB) is involved in expanded polyglutamine-induced toxicity.

51 Aggregation of expanded polyglutamine is thought to be a common patholo

52 The age of onset depended on an expanded polyglutamine length; phenotype severity correl

53 How the huntingtin protein with expanded polyglutamines (mutant huntingtin) causes the d

54 hese mutations for interactions with tau and expanded-polyglutamine overexpression and found a few ca

55 ific degeneration when mutated to contain an expanded polyglutamine (poly(Q)) domain.

56 Huntington disease (HD) is caused by an expanded polyglutamine (poly(Q)) repeat near the N termi

57 own neurodegenerative disorders caused by an expanded polyglutamine (poly(Q)) tract in the disease pr

58 ization were also activated by expression of expanded polyglutamine (poly-Q) proteins SCA1, SCA3, and

59 al other neurological diseases are caused by expanded polyglutamine [poly(Gln)] tracts in different p

60 Here we have used exon 1 of the HD gene with expanded polyglutamine [poly(Q)] repeats and enhanced gr

61 own neurodegenerative disorders caused by an expanded polyglutamine [poly(Q)] tract in the disease pr

62 of observations point to the aggregation of expanded polyglutamine [poly(Q)]-containing proteins as

63 The formation of amyloid-like aggregates by expanded polyglutamine (polyGln) sequences is suspected

64 late aggregation and nuclear localization of expanded polyglutamine polypeptides derived from the and

65 diseases resulting from proteins containing expanded polyglutamine (polyQ) are characteristically as

66 Mutant huntingtin (mHtt) with expanded polyglutamine (polyQ) binds to p53 and upregula

67 s expressing mutant huntingtin (htt) with an expanded polyglutamine (polyQ) domain are useful for stu

68 etic neurodegenerative disorder caused by an expanded polyglutamine (polyQ) domain near the N-terminu

69 nce the toxic effect of polypeptides with an expanded polyglutamine (polyQ) domain.

70 misfolding of huntingtin (htt) caused by an expanded polyglutamine (polyQ) domain.

71 ses, such IBs can be formed by proteins with expanded polyglutamine (polyQ) domains (e.g., huntingtin

72 In Saccharomyces cerevisae, expanded polyglutamine (polyQ) fragments are assembled i

73 Huntington's disease (HD) arises from an expanded polyglutamine (polyQ) in the N-terminus of the

74 Mutant HTT with expanded polyglutamine (polyQ) is widely expressed in th

75 e strand antisense to JPH3, which encodes an expanded polyglutamine (polyQ) protein.

76 to monitor the diverse biophysical states of expanded polyglutamine (polyQ) proteins expressed in Cae

77 Expanded polyglutamine (polyQ) proteins in Huntington's

78 Ataxin-3 protein with an expanded polyglutamine (polyQ) repeat causes spinocerebe

79 dates for the toxic molecular species in the expanded polyglutamine (polyQ) repeat diseases range fro

80 s and is characterized by the presence of an expanded polyglutamine (polyQ) repeat in the huntingtin

81 nset neurodegenerative disorder caused by an expanded polyglutamine (polyQ) repeat in the TATA-box-bi

82 the CAG repeat in the HTT gene, encoding an expanded polyglutamine (polyQ) repeat within the HTT pro

83 is a neurodegenerative disorder caused by an expanded polyglutamine (polyQ) repeat within the protein

84 Although expanded polyglutamine (polyQ) repeats are inherently to

85 Aggregation of proteins containing expanded polyglutamine (polyQ) repeats is the cytopathol

86 gregation of huntingtin protein arising from expanded polyglutamine (polyQ) sequences in the exon-1 r

87 An expanded polyglutamine (polyQ) stretch in the protein hu

88 on protects neurons from the early phases of expanded polyglutamine (polyQ) toxicity, and this protec

89 ractions are enhanced in the presence of the expanded polyglutamine (polyQ) tract and are stronger in

90 Mutant huntingtin protein (mHTT) contains an expanded polyglutamine (polyQ) tract and causes Huntingt

91 aggresomes induced by proteins containing an expanded polyglutamine (polyQ) tract are pathologic hall

92 Fragments of proteins containing an expanded polyglutamine (polyQ) tract are thought to init

93 The expanded polyglutamine (polyQ) tract form of ataxin-1 dr

94 n the huntingtin (HTT) gene, resulting in an expanded polyglutamine (polyQ) tract in HTT protein.

95 is a neurodegenerative disorder caused by an expanded polyglutamine (polyQ) tract in the huntingtin (

96 Huntington's disease (HD) is caused by an expanded polyglutamine (polyQ) tract in the huntingtin (

97 XN3 lacking catalytic activity or bearing an expanded polyglutamine (polyQ) tract led to partially ov

98 ELISA-based methods use Abs that target the expanded polyglutamine (polyQ) tract to quantify mutant

99 ce of the ATXN3 gene, and this results in an expanded polyglutamine (polyQ) tract within the Ataxin-3

100 ting neurodegenerative disorder caused by an expanded polyglutamine (polyQ) tract within the huntingt

101 pathologic mutant HTT (mHTT) protein with an expanded polyglutamine (polyQ) tract.

102 ssemblies of huntingtin (HTT) fragments with expanded polyglutamine (polyQ) tracts are a pathological

103 Expanded polyglutamine (polyQ) tracts are associated wit

104 Proteins with expanded polyglutamine (polyQ) tracts have been linked t

105 Expanded polyglutamine (polyQ) tracts have been linked t

106 ine neurodegenerative diseases are caused by expanded polyglutamine (polyQ) tracts in different prote

107 The aggregation of proteins with expanded polyglutamine (polyQ) tracts is directly releva

108 seases caused by misfolding of proteins with expanded polyglutamine (polyQ) tracts, such as Huntingto

109 haracterized by aggregation of proteins with expanded polyglutamine (polyQ) tracts.

110 caused by the toxic effects triggered by an expanded polyglutamine (polyQ) within Atxn1 resulting in

111 rin-gamma3/AGAP3, facilitated degradation of expanded polyglutamine protein (polyQ) via the nuclear u

112 Changes caused by an expanded polyglutamine protein are possibly influenced b

113 Mutant ataxin-1, the expanded polyglutamine protein causing spinocerebellar a

114 nhances parkin binding and ubiquitination of expanded polyglutamine protein in vitro suggesting that

115 al markedly divergent mobility states for an expanded polyglutamine protein, ataxin-3, and establish

116 Parkin forms a complex with the expanded polyglutamine protein, heat shock protein 70 (H

117 may be important for the elimination of the expanded polyglutamine protein.

118 tes the ubiquitination and degradation of an expanded polyglutamine protein.

119 ment and caspase-12 activation induced by an expanded polyglutamine protein.

120 presence of inclusions or aggregates of the expanded polyglutamine protein.

121 Expanded polyglutamine proteins accumulate abnormally in

122 ive disorders, but the dynamic properties of expanded polyglutamine proteins are poorly understood.

123 hus, aggregation and nuclear localization of expanded polyglutamine proteins are regulated cellular p

124 The expanded polyglutamine proteins are ubiquitously express

125 eceptor (wtGR) suppressed the aggregation of expanded polyglutamine proteins derived from AR and hunt

126 Expanded polyglutamine proteins form aggregates, includi

127 Huntingtin aggregates sequester other expanded polyglutamine proteins in the cytoplasm and lea

128 an experimental framework to understand why expanded polyglutamine proteins may be toxic only to cer

129 que protein associations or conformations of expanded polyglutamine proteins may determine subsequent

130 that some of the gene expression effects of expanded polyglutamine proteins occur independently of p

131 Chronic exposure of cells to expanded polyglutamine proteins results in eventual cell

132 aggregation and nuclear localization of the expanded polyglutamine proteins.

133 be responsible for the pathogenic effects of expanded polyglutamine proteins.

134 eristic of the aggregation pathway for toxic expanded polyglutamine proteins.

135 edistributed into inclusions formed by three expanded polyglutamine proteins: a pathologic ataxin-3 f

136 lular accumulation of mutant Huntingtin with expanded polyglutamine provides a context-dependent cyto

137 urodegenerative disorder that arises from an expanded polyglutamine region in the N terminus of the H

138 Huntington disease results from an expanded polyglutamine region in the N terminus of the h

139 gation of huntingtin exon-1 (Httex1) with an expanded polyglutamine region is a key pathological hall

140 on of amino-terminal fragments containing an expanded polyglutamine region.

141 e death of primary rat neurons induced by an expanded polyglutamine repeat (Q79).

142 Neither normal nor expanded polyglutamine repeat alone interacted with CBS

143 atal neurodegenerative disorder caused by an expanded polyglutamine repeat in huntingtin (HTT) protei

144 nant neurodegenerative disorder caused by an expanded polyglutamine repeat in the huntingtin gene.

145 Targeted expression of the protein with an expanded polyglutamine repeat led to nuclear inclusion (

146 Expression of an expanded polyglutamine repeat within the Huntingtin (Htt

147 s, including Huntingtin exon 1 containing an expanded polyglutamine repeat, aggregate faster under th

148 xpressing exon 1 of huntingtin containing an expanded polyglutamine repeat, altered the course of the

149 Aggregation of expanded polyglutamine repeat-containing fragments of th

150 ant form of Huntingtin protein containing an expanded polyglutamine repeat.

151 Because in vitro expanded polyglutamine repeats are glutaminyl-donor subs

152 Proteins with expanded polyglutamine repeats cause Huntington's diseas

153 occurs in vivo in response to expression of expanded polyglutamine repeats has not been tested.

154 Expanded polyglutamine repeats have been proposed to cau

155 herited neurodegenerative disorder caused by expanded polyglutamine repeats in the huntingtin (Htt) p

156 ase (HD) is a rare genetic disease caused by expanded polyglutamine repeats in the huntingtin protein

157 ion functions, and promotes the clearance of expanded polyglutamine repeats in vivo and in vitro.

158 he gene encoding huntingtin (Htt) leading to expanded polyglutamine repeats of mutant Htt (mHtt) that

159 Expanded polyglutamine repeats specifically interfere wi

160 lding and aggregation of proteins containing expanded polyglutamine repeats underlie Huntington's dis

161 with the aggregation of proteins containing expanded polyglutamine sequences.

162 ect interaction with mutant Htt, because the expanded polyglutamine stretch and adjacent proline-rich

163 s disease (HD) is initiated by an abnormally expanded polyglutamine stretch in the huntingtin protein

164 ncoding a version of the Htt protein with an expanded polyglutamine stretch.

165 determinant in the biochemical properties of expanded polyglutamine that are central to their chronic

166 An expanded polyglutamine tract (>37 glutamines) in the N-t

167 aggregation of the androgen receptor with an expanded polyglutamine tract (AR-polyQ) has been linked

168 pressing a human huntingtin fragment with an expanded polyglutamine tract (Htn-Q150).

169 huntingtin's protein exon-1 fragment with an expanded polyglutamine tract (Htt-103Q), which is depend

170 cterized by misfolding and aggregation of an expanded polyglutamine tract (polyQ).

171 N-terminal huntingtin fragments with an expanded polyglutamine tract aberrantly localized to int

172 In contrast, Drosophila expressing an expanded polyglutamine tract alone, or an expanded polyg

173 A gain of toxic function as a result of an expanded polyglutamine tract can cause the protein hunti

174 Here we show that the expanded polyglutamine tract differentially affects the

175 rotein aggregation diseases is an abnormally expanded polyglutamine tract found in the respective pro

176 ington's disease (HD), which is caused by an expanded polyglutamine tract in huntingtin (htt).

177 It is caused by an expanded polyglutamine tract in huntingtin (Htt).

178 ssive neurodegenerative disease caused by an expanded polyglutamine tract in huntingtin protein (Htt)

179 an expanded polyglutamine tract alone, or an expanded polyglutamine tract in the context of the spino

180 the Huntingtin (HTT) gene, translating to an expanded polyglutamine tract in the HTT protein.

181 is a neurodegenerative disorder caused by an expanded polyglutamine tract in the huntingtin (HTT) pro

182 Huntington disease derives from a critically expanded polyglutamine tract in the huntingtin (Htt) pro

183 on in the huntingtin gene that results in an expanded polyglutamine tract in the huntingtin protein.

184 etic neurodegenerative disorder caused by an expanded polyglutamine tract in the huntingtin protein.

185 Huntington's disease (HD) is caused by an expanded polyglutamine tract in the huntingtin protein.

186 The resulting expanded polyglutamine tract in the N-terminal region of

187 Huntington's disease (HD) is caused by an expanded polyglutamine tract in the protein huntingtin (

188 In addition, the presence of an expanded polyglutamine tract in the SBMA androgen recept

189 inant neurodegenerative disease caused by an expanded polyglutamine tract in the ubiquitously express

190 he pathway to neuronal dysfunction, while an expanded polyglutamine tract is essential for neuronal d

191 In SCA1, the expanded polyglutamine tract is in the ataxin-1 (ATXN1)

192 ion of the mutant huntingtin protein with an expanded polyglutamine tract plays a central role in the

193 ed on identifying the mechanism by which the expanded polyglutamine tract renders a protein toxic to

194 es of mutant huntingtin exon 1 containing an expanded polyglutamine tract with 51 residues (mhttQ51),

195 rmal CAG expansion, which translates into an expanded polyglutamine tract within ataxin-3.

196 ataxin-1 SUMOylation in the presence of the expanded polyglutamine tract, ataxin-1[82Q].

197 sfolding of huntingtin (HTT) protein with an expanded polyglutamine tract, could also benefit from th

198 by exon 1 of the htt gene and containing an expanded polyglutamine tract, form fibrils that accumula

199 coded by exon-1 (htt(ex1)) and containing an expanded polyglutamine tract, forms fibrils that accumul

200 ical features caused by ATXN1[82Q] having an expanded polyglutamine tract, they fail to manifest the

201 The expansion results in an expanded polyglutamine tract, which likely confers a nov

202 plasmids expressing a transcript encoding an expanded polyglutamine tract.

203 pression of mutant ataxin-1 that contains an expanded polyglutamine tract.

204 lve interactions with other proteins via the expanded polyglutamine tract.

205 gene, yielding a Huntingtin protein with an expanded polyglutamine tract.

206 generation requires expressing ATXN1 with an expanded polyglutamine tract.

207 with disease caused by ATXN1[82Q] having an expanded polyglutamine tract.

208 Previous studies suggest that expanded polyglutamine tracts alter transcription by seq

209 asm, N-terminal fragments of huntingtin with expanded polyglutamine tracts are able to accumulate in

210 Expanded polyglutamine tracts are responsible for at lea

211 Expanded polyglutamine tracts cause huntingtin and other

212 d a direct viral approach to locally express expanded polyglutamine tracts fused to the green fluores

213 It has been suggested that proteins with expanded polyglutamine tracts impair ubiquitin-dependent

214 f CHIP to protect against toxicity caused by expanded polyglutamine tracts in different protein conte

215 Aggregation of expanded polyglutamine tracts is associated with nine di

216 otoxicity induced by Htt proteins containing expanded polyglutamine tracts is likely mediated, at lea

217 This flexibility is impaired with expanded polyglutamine tracts, and we can detect changes

218 Here we show that polypeptides containing expanded polyglutamine tracts, but not normal N-terminal

219 caused by expression of proteins containing expanded polyglutamine tracts.

220 ed in other transgenic models overexpressing expanded polyglutamine tracts.

221 ucts containing SBMA or DRPLA with normal or expanded polyglutamine tracts.

222 neurodegenerative disorders associated with expanded polyglutamine tracts.

223 Although expanded polyglutamine triggers disease, functional prop