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 In mouse models, proteins with expanded polyglutamine cause transcriptional dysregulati

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

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

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

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

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

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

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

21 An expanded polyglutamine domain in huntingtin underlies th

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

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

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

25 Expanded polyglutamine domain proteins possess propertie

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

27 Abnormally expanded polyglutamine domains are associated with at le

28 Proteins with expanded polyglutamine domains cause eight inherited neu

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

30 Abnormally expanded polyglutamine domains in proteins are associate

31 Abnormally expanded polyglutamine domains in proteins are associate

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

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

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

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

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

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

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

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

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

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

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

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

44 Because the expression of expanded polyglutamine in selected neuronal populations

45 Expanded polyglutamine induced a marked increase in expr

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

73 Expanded polyglutamine (polyQ) proteins in Huntington's

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

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

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

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

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

79 Although expanded polyglutamine (polyQ) repeats are inherently to

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

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

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

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

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

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

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

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

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

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

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

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

92 Expanded polyglutamine (polyQ) tracts are associated wit

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

94 Expanded polyglutamine (polyQ) tracts have been linked t

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

110 Expanded polyglutamine proteins accumulate abnormally in

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

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

113 The expanded polyglutamine proteins are ubiquitously express

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

115 Expanded polyglutamine proteins form aggregates, includi

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

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

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

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

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

121 aggregation and nuclear localization of the expanded polyglutamine proteins.

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

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

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

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

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

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

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

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

130 Neither normal nor expanded polyglutamine repeat alone interacted with CBS

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

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

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

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

135 Aggregation of expanded polyglutamine repeat-containing fragments of th

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

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

138 Proteins with expanded polyglutamine repeats cause Huntington's diseas

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

140 Expanded polyglutamine repeats have been proposed to cau

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

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

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

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

145 Expanded polyglutamine repeats specifically interfere wi

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

147 with the aggregation of proteins containing expanded polyglutamine sequences.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

183 plasmids expressing a transcript encoding an expanded polyglutamine tract.

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

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

186 generation requires expressing ATXN1 with an expanded polyglutamine tract.

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

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

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

190 Expanded polyglutamine tracts are responsible for at lea

191 Expanded polyglutamine tracts cause huntingtin and other

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

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

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

195 Aggregation of expanded polyglutamine tracts is associated with nine di

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

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

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

199 caused by expression of proteins containing expanded polyglutamine tracts.

200 ed in other transgenic models overexpressing expanded polyglutamine tracts.

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

202 neurodegenerative disorders associated with expanded polyglutamine tracts.

203 Although expanded polyglutamine triggers disease, functional prop