ライフサイエンスコーパス: polyglutamine tract

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

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

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

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

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

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

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

8 expressing a transcript encoding an expanded polyglutamine tract.

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

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

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

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

13 generative diseases caused by expansion of a polyglutamine tract.

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

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

16 results in a mutant protein with an extended polyglutamine tract.

17 iated multimerization involving the ataxin-1 polyglutamine tract.

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

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

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

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

22 actions with other proteins via the expanded polyglutamine tract.

23 xon 1 CAG repeat that lengthens huntingtin's polyglutamine tract.

24 elding a Huntingtin protein with an expanded polyglutamine tract.

25 n requires expressing ATXN1 with an expanded polyglutamine tract.

26 1-17 domains and possibly with the proximal polyglutamine tract.

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

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

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

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

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

32 nteraction motifs neighboring the pathogenic polyglutamine tract.

33 known result from CAG expansions that encode polyglutamine tracts.

34 e diseases caused by pathogenic expansion of polyglutamine tracts.

35 y expression of proteins containing expanded polyglutamine tracts.

36 o suppress aggregation of proteins with long polyglutamine tracts.

37 er transgenic models overexpressing expanded polyglutamine tracts.

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

39 motes self-association and nucleation of the polyglutamine tracts.

40 ntingtin proteins containing short and large polyglutamine tracts.

41 enerative disorders associated with expanded polyglutamine tracts.

42 ontaining exon 1 of huntingtin with extended polyglutamine tracts.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

59 Expanded polyglutamine tracts are responsible for at least eight

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

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

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

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

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

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

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

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

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

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

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

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

72 Expanded polyglutamine tracts cause huntingtin and other proteins

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

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

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

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

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

78 The expansion of polyglutamine tracts encoded by CAG trinucleotide repeat

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

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

81 s disease (HD), a prototypic NDD caused by a polyglutamine tract expansion in huntingtin (HTT), which

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

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

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

85 Pathways leading from polyglutamine tract expansion to disease pathogenesis re

86 man neurodegenerative disease resulting from polyglutamine tract expansion.

87 ited neurodegenerative disorders caused by a polyglutamine tract expansion.

88 herited neurodegenerative diseases caused by polyglutamine tract expansion.

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

90 1 of the htt gene and containing an expanded polyglutamine tract, form fibrils that accumulate within

91 exon-1 (htt(ex1)) and containing an expanded polyglutamine tract, forms fibrils that accumulate in ne

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

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

94 ighly aggregation-prone protein containing a polyglutamine tract >35 repeats (mHTT) that has been sho

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

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

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

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

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

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

101 1 (SCA1), a disease caused by expansion of a polyglutamine tract in ataxin 1 (ATXN1).

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

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

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

105 axia type 1 is caused by an expansion of the polyglutamine tract in ATAXIN-1.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

137 ngtin (HTT) gene, translating to an expanded polyglutamine tract in the HTT protein.

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

139 odegenerative disorder caused by an expanded polyglutamine tract in the huntingtin (HTT) protein.

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

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

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

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

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

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

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

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

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

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

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

151 huntingtin gene (HTT), resulting in a toxic polyglutamine tract in the huntingtin protein.

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

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

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

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

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

157 he mutant gene, ATXN3, producing an enlarged polyglutamine tract in the mutant protein.

158 Expansion of the polyglutamine tract in the N terminus of Ataxin-1 is the

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

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

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

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

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

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

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

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

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

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

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

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

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

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

173 to specific depletion of proteins containing polyglutamine tracts including core-binding factor alpha

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

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

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

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

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

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

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

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

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

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

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

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

186 with (htt(NT)Q(10)) a ten-residue C-terminal polyglutamine tract, is investigated by NMR spectroscopy

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

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

189 es cerevisiae, and we propose that different polyglutamine tract lengths may be adaptive within certa

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

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

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

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

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

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

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

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

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

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

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

201 esidue N-terminal amphiphilic domain (NT), a polyglutamine tract of variable length (Q(n)), and a pol

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

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

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

205 ington's disease results from expansion of a polyglutamine tract (polyQ) in mutant huntingtin (mHTT)

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

207 Polyglutamine tracts promote protein aggregation in vitr

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

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

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

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

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

213 ss this sequence feature and the tendency of polyglutamine tracts to vary in length among strains of

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

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

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

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

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

219 d forms of the huntingtin protein containing polyglutamine tracts with more than 36 repeats.

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

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

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

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

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

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

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

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

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

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

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

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