ライフサイエンスコーパス: polyglutamine tract

1 Q81, which consists of multiple repeats of a polyglutamine tract.

2 which has a conserved AXH domain but lacks a polyglutamine tract.

3 cleotide (CAG) repeat coding for an extended polyglutamine tract.

4 n that is translated into an abnormally long polyglutamine tract.

5 elix, a leucine zipper, a zinc finger, and a polyglutamine tract.

6 lying mutation is a CAG expansion encoding a polyglutamine tract.

7 expressing a transcript encoding an expanded polyglutamine tract.

8 ne is increased, resulting in expansion of a polyglutamine tract.

9 lying mutation is a CAG expansion encoding a polyglutamine tract.

10 n that is translated into an abnormally long polyglutamine tract.

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

12 generative diseases caused by expansion of a polyglutamine tract.

13 ot caused by a CAG expansion translated as a polyglutamine tract.

14 n of the androgen receptor gene coding for a polyglutamine tract.

15 iated multimerization involving the ataxin-1 polyglutamine tract.

16 rative disorders caused by an expansion of a polyglutamine tract.

17 appear to be influenced by expansion of the polyglutamine tract.

18 by the expansion of a CAG repeat encoding a polyglutamine tract.

19 o GAPDH does not vary with the length of the polyglutamine tract.

20 actions with other proteins via the expanded polyglutamine tract.

21 n requires expressing ATXN1 with an expanded polyglutamine tract.

22 1-17 domains and possibly with the proximal polyglutamine tract.

23 K6 to F17, i.e., up to the very start of the polyglutamine tract.

24 ease caused by ATXN1[82Q] having an expanded polyglutamine tract.

25 in regions of the protein in addition to the polyglutamine tract.

26 t repeat stretch, which encodes an elongated polyglutamine tract.

27 ion of a CAG trinucleotide repeat encoding a polyglutamine tract.

28 nteraction motifs neighboring the pathogenic polyglutamine tract.

29 t disorders are linked to the expansion of a polyglutamine tract.

30 o suppress aggregation of proteins with long polyglutamine tracts.

31 known result from CAG expansions that encode polyglutamine tracts.

32 e diseases caused by pathogenic expansion of polyglutamine tracts.

33 y expression of proteins containing expanded polyglutamine tracts.

34 er transgenic models overexpressing expanded polyglutamine tracts.

35 aining SBMA or DRPLA with normal or expanded polyglutamine tracts.

36 enerative disorders associated with expanded polyglutamine tracts.

37 ontaining exon 1 of huntingtin with extended polyglutamine tracts.

38 rminal huntingtin fragments with an expanded polyglutamine tract aberrantly localized to intracellula

39 Expansion of the polyglutamine tract above 35 repeats causes disease, wit

40 contrast, Drosophila expressing an expanded polyglutamine tract alone, or an expanded polyglutamine

41 Hypothesizing that long polyglutamine tracts alter gene expression, we found cer

42 Previous studies suggest that expanded polyglutamine tracts alter transcription by sequestering

43 the proteins with a process dictated by the polyglutamine tracts, although increasing evidence sugge

44 a putative transcription factor containing a polyglutamine tract and a forkhead DNA-binding domain, i

45 ding for a transcription factor containing a polyglutamine tract and a forkhead DNA-binding domain.

46 encodes a transcription factor containing a polyglutamine tract and a forkhead domain.

47 scoideum has evolved to normally encode long polyglutamine tracts and express these proteins in a sol

48 ave been reported to cleave only once within polyglutamine tracts and then only after the N-terminal

49 ER; binding is modulated by the size of the polyglutamine tract, and mutating a sequence adjacent to

50 This flexibility is impaired with expanded polyglutamine tracts, and we can detect changes in hunti

51 on of the androgen receptor with an expanded polyglutamine tract (AR-polyQ) has been linked to the de

52 covery that residues in ATXN1 outside of the polyglutamine tract are crucial for pathogenesis hinted

53 rminal fragments of huntingtin with expanded polyglutamine tracts are able to accumulate in the nucle

54 Expanded polyglutamine tracts are responsible for at least eight

55 The proteins harbouring these polyglutamine tracts are unrelated and without exception

56 is due to an androgen receptor containing a polyglutamine tract (ARpolyQ) that misfolds and aggregat

57 ceptor sites; human MPI is translated into a polyglutamine tract associated with spinocerebellar atax

58 expansion that results in elongation of the polyglutamine tract at the N terminus of huntingtin (Htt

59 isease (HD) is caused by an expansion of the polyglutamine tract at the N terminus of huntingtin.

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

61 The WW domain belonging to polyglutamine tract-binding protein 1 (PQBP1) is of part

62 Polyglutamine tract-binding protein-1 (PQBP-1) is a 265-

63 Drosophila contains many proteins with polyglutamine tracts but these are generally encoded by

64 e show that polypeptides containing expanded polyglutamine tracts, but not normal N-terminal huntingt

65 Our data support the hypothesis that the polyglutamine tract can act as a flexible domain, allowi

66 of toxic function as a result of an expanded polyglutamine tract can cause the protein huntingtin to

67 Expanded polyglutamine tracts cause huntingtin and other proteins

68 an 897 amino acid protein with an expandable polyglutamine tract close to its N-terminus.

69 peats are translated into an abnormally long polyglutamine tract close to the N-terminus of the HD ge

70 of huntingtin (HTT) protein with an expanded polyglutamine tract, could also benefit from this approa

71 Here we show that the expanded polyglutamine tract differentially affects the function

72 sis derives, at least in part, from the long polyglutamine tract encoded by mutant HTT.

73 The expansion of polyglutamine tracts encoded by CAG trinucleotide repeat

74 a stretch of glutamine amino acid residues (polyglutamine tract) encoded by any of several SCA-causi

75 These studies demonstrate that polyglutamine tract expansion and localization of ataxin

76 Huntington's disease (HD) is caused by a polyglutamine tract expansion in huntingtin (HTT).

77 sive motor neuron disease caused by abnormal polyglutamine tract expansion in the androgen receptor (

78 erebellar ataxia type 17 that is caused by a polyglutamine tract expansion in the TATA box-binding pr

79 Pathways leading from polyglutamine tract expansion to disease pathogenesis re

80 man neurodegenerative disease resulting from polyglutamine tract expansion.

81 ited neurodegenerative disorders caused by a polyglutamine tract expansion.

82 herited neurodegenerative diseases caused by polyglutamine tract expansion.

83 NTFs typically encompass a polyglutamine tract flanked by an N-terminal 17-residue

84 gregation diseases is an abnormally expanded polyglutamine tract found in the respective proteins.

85 t viral approach to locally express expanded polyglutamine tracts fused to the green fluorescent prot

86 An expanded polyglutamine tract (>37 glutamines) in the N-terminal r

87 Additionally, the sequence context of a polyglutamine tract has recently been shown to modulate

88 udy suggested that the glutamine residues in polyglutamine tracts have a significant propensity to ad

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

90 n's protein exon-1 fragment with an expanded polyglutamine tract (Htt-103Q), which is dependent upon

91 s been suggested that proteins with expanded polyglutamine tracts impair ubiquitin-dependent proteoly

92 1 (SCA1), a disease caused by expansion of a polyglutamine tract in ataxin 1 (ATXN1).

93 by the expansion of a CAG repeat encoding a polyglutamine tract in Ataxin-1 (ATXN1).

94 ion analysis excluded the involvement of the polyglutamine tract in ataxin-1 self-association, and in

95 generative disorder caused by expansion of a polyglutamine tract in ataxin-1.

96 ia type 2 (SCA2) is caused by expansion of a polyglutamine tract in ataxin-2, a protein of unknown fu

97 by the expansion of a CAG repeat encoding a polyglutamine tract in ataxin-2, the SCA2 gene product.

98 Expansion of a polyglutamine tract in ataxin-3 (AT3) results in spinoce

99 ansion of a translated CAG repeat encoding a polyglutamine tract in ataxin-7, the SCA7 gene product.

100 generative disorder caused by expansion of a polyglutamine tract in ATXN1.

101 used by expansion of a CAG repeat encoding a polyglutamine tract in ATXN7, a component of the SAGA hi

102 ility in Huntington's disease (HD) encodes a polyglutamine tract in full-length huntingtin that is co

103 disease (HD), which is caused by an expanded polyglutamine tract in huntingtin (htt).

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

105 Thus, the polyglutamine tract in huntingtin appears to regulate mi

106 rodegenerative disease caused by an expanded polyglutamine tract in huntingtin protein (Htt).

107 t causes Huntington's disease (HD) encodes a polyglutamine tract in huntingtin, which first targets t

108 TBP contains a polymorphic polyglutamine tract in its N-terminal region, and expans

109 y of expanded HD CAG repeats that encode the polyglutamine tract in mutant huntingtin has been implic

110 the Huntington's disease gene HTT extends a polyglutamine tract in mutant huntingtin that enhances i

111 of an involvement of a region other than the polyglutamine tract in polyglutamine pathologies.

112 by expansion of a CAG repeat that encodes a polyglutamine tract in the affected protein.

113 Expansion of a polyglutamine tract in the androgen receptor (AR) causes

114 egenerative disease caused by expansion of a polyglutamine tract in the androgen receptor (AR).

115 rative disease caused by an expansion of the polyglutamine tract in the androgen receptor (AR).

116 of a CAG triplet repeat sequence encoding a polyglutamine tract in the androgen receptor.

117 generative disorder caused by expansion of a polyglutamine tract in the ataxin-7 protein.

118 is associated with anomalous expansion of a polyglutamine tract in the coding region of the correspo

119 ed polyglutamine tract alone, or an expanded polyglutamine tract in the context of the spinocerebella

120 generative diseases caused by expansion of a polyglutamine tract in the disease protein, in this case

121 generative diseases caused by expansion of a polyglutamine tract in the disease protein.

122 used by expansion of a CAG repeat encoding a polyglutamine tract in the disease protein.

123 isorder associated with the expansion of the polyglutamine tract in the exon-1 domain of the huntingt

124 SBMA is caused by expansions of a polyglutamine tract in the gene coding for androgen rece

125 This results in the lengthening of a polyglutamine tract in the gene product ataxin-1.

126 n the huntingtin (HTT) gene, which encodes a polyglutamine tract in the HTT protein.

127 It is caused by the expansion of a polyglutamine tract in the huntingtin (HTT) protein, whi

128 caused by a CAG repeat expansion encoding a polyglutamine tract in the huntingtin (Htt) protein.

129 disorder, is caused by a lengthening of the polyglutamine tract in the huntingtin (Htt) protein.

130 a CAG trinucleotide sequence that encodes a polyglutamine tract in the huntingtin (Htt) protein.

131 n disease derives from a critically expanded polyglutamine tract in the huntingtin (Htt) protein; a s

132 exon 1 of the HTT gene that translates to a polyglutamine tract in the huntingtin protein (HTT).

133 Expansion of a polyglutamine tract in the huntingtin protein causes neu

134 Expansion of the polyglutamine tract in the huntingtin protein results in

135 generative disorder caused by expansion of a polyglutamine tract in the huntingtin protein that resul

136 a CAG repeat expansion encoding an extended polyglutamine tract in the huntingtin protein.

137 egenerative disease caused by expansion of a polyglutamine tract in the huntingtin protein.

138 by the expansion of CAG repeats coding for a polyglutamine tract in the huntingtin protein.

139 ative disorder resulting from expansion of a polyglutamine tract in the Huntingtin protein.

140 odegenerative disorder caused by an expanded polyglutamine tract in the huntingtin protein.

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

142 However, there was no extended polyglutamine tract in the mouse SCA2 cDNA, suggesting t

143 disease (HD) is caused by the expansion of a polyglutamine tract in the N-terminal region of huntingt

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

145 , this expansion produces an abnormally long polyglutamine tract in the protein ataxin-1.

146 is one such disease-caused by expansion of a polyglutamine tract in the protein ataxin-1.

147 Mutations resulting in the expansion of a polyglutamine tract in the protein ataxin-2 give rise to

148 generative disorder caused by expansion of a polyglutamine tract in the protein huntingtin (Htt), whi

149 gton's disease (HD) is caused by an expanded polyglutamine tract in the protein huntingtin (htt).

150 In addition, the presence of an expanded polyglutamine tract in the SBMA androgen receptor appear

151 rodegenerative disease caused by an expanded polyglutamine tract in the ubiquitously expressed huntin

152 protect against toxicity caused by expanded polyglutamine tracts in different protein contexts.

153 dysregulation occurs in yeast, we expressed polyglutamine tracts in Saccharomyces cerevisiae.

154 generative disease caused by an expansion of polyglutamine tracts in the cytosolic protein ataxin-2 (

155 of which are caused by CAG repeats encoding polyglutamine tracts in the disease gene protein.

156 untingtin (amino acids 1-171), including the polyglutamine tract, in the yeast two-hybrid system.

157 this binding diminishes as the length of its polyglutamine tract increases.

158 act, and mutating a sequence adjacent to the polyglutamine tract inhibits the AT3-VCP interaction and

159 tin is an essential protein that with mutant polyglutamine tracts initiates dominant striatal neurode

160 ence of in vivo recruitment of proteins with polyglutamine tracts into intraneuronal aggregates.

161 function in the context of expansion of the polyglutamine tract is critical for understanding the di

162 y to neuronal dysfunction, while an expanded polyglutamine tract is essential for neuronal death.

163 In SCA1, the expanded polyglutamine tract is in the ataxin-1 (ATXN1) protein.

164 One explanation is that the polyglutamine tract is not the only determinant of neuro

165 This polyglutamine tract is thought to confer a toxic gain of

166 Aggregation of expanded polyglutamine tracts is associated with nine different n

167 induced by Htt proteins containing expanded polyglutamine tracts is likely mediated, at least in par

168 gic disorder produced by expansion of an Htt polyglutamine tract, is characterized by neurodegenerati

169 vely worsened with age and was influenced by polyglutamine tract length in mutant huntingtin (mhtt).

170 nd explains the positive correlation between polyglutamine tract length, protein aggregation, and dis

171 ic mice containing pathological alleles with polyglutamine tract lengths of 64, 67, 72, 76 and 84 rep

172 es of disease-specific proteins that contain polyglutamine tracts longer than a critical length.

173 Lys(16), Lys(194) preceding the polyglutamine tract, Lys(610)/Lys(697) in the C-terminal

174 RNA metabolism and that the expansion of the polyglutamine tract may alter this function.

175 the causative mutation is expansion of a CAG/polyglutamine tract near the 5' end of the androgen rece

176 n's disease (HD) is caused by expansion of a polyglutamine tract near the N-terminal of huntingtin.

177 aggregation is the anomalous expansion of a polyglutamine tract near the protein N-terminus, but the

178 expansion of variable length, which encodes polyglutamine tracts near the amino terminus of the HD p

179 uman huntingtin fragment containing a mutant polyglutamine tract of 103Q fused to green fluorescent p

180 ownstream polyproline region that flanks the polyglutamine tract of huntingtin.

181 e the molecular basis of HD, we extended the polyglutamine tract of the mouse homologue, Hdh, by targ

182 In addition, we find that polyglutamine tracts of increasing length are associated

183 e mutant huntingtin protein with an expanded polyglutamine tract plays a central role in the patholog

184 s directly located upstream of the protein's polyglutamine tract, plays a decisive role in several im

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

186 Polyglutamine tracts promote protein aggregation in vitr

187 ntifying the mechanism by which the expanded polyglutamine tract renders a protein toxic to a subset

188 actions with other proteins containing short polyglutamine tracts such as the transcriptional coactiv

189 ndary structure accompanies expansion of the polyglutamine tract, suggesting destabilization of the n

190 in the Huntingtin gene, creating an extended polyglutamine tract that makes the Huntingtin protein to

191 ures caused by ATXN1[82Q] having an expanded polyglutamine tract, they fail to manifest the age-relat

192 ssociation is modulated by the length of the polyglutamine tract whereby S4 shows a stronger associat

193 The C-terminal fragment bears a polyglutamine tract which, when expanded (Q33) as in spi

194 protein of unknown function that contains a polyglutamine tract, which is expanded in patients with

195 The expansion results in an expanded polyglutamine tract, which likely confers a novel, toxic

196 ant huntingtin exon 1 containing an expanded polyglutamine tract with 51 residues (mhttQ51), and reso

197 Expansion of a polyglutamine tract within ataxin-1 causes spinocerebell

198 expansion, which translates into an expanded polyglutamine tract within ataxin-3.

199 neuron disease caused by the expansion of a polyglutamine tract within the androgen receptor.

200 ar ataxia associated with the expansion of a polyglutamine tract within the ataxin-1 (ATXN1) protein.

201 riplet in the ATXN3 gene, translating into a polyglutamine tract within the ataxin-3 protein.

202 generative disorder caused by expansion of a polyglutamine tract within the ataxin-7 protein.

203 Disease (HD) is caused by an expansion of a polyglutamine tract within the huntingtin (htt) protein.

204 rative disorder caused by the expansion of a polyglutamine tract within the MJD1 gene.

205 t AR function is reduced by expansion of the polyglutamine tract within the receptor [AR(Gln)(n)]; th

206 rative disorder caused by the expansion of a polyglutamine tract within the SCA1 gene product, ataxin

207 erative disease caused by the expansion of a polyglutamine tract within the SCA1 product, ataxin-1.

208 refore, we hypothesize that wild-type length polyglutamine tracts within huntingtin can form a flexib