ライフサイエンスコーパス: polyglutamine

1 same JUNQ-like inclusion whereas the other, polyglutamine (72Q), formed spatially distinct IPOD-like

2 lins by genetic ablation or sequestration in polyglutamine aggregates leads to accumulation of non-in

3 ve disorders and intracerebral deposition of polyglutamine aggregates motivates attempts to better un

4 Furthermore, we found that polyglutamine aggregates specifically recruit some stres

5 lerance and reduced levels of stress-induced polyglutamine aggregates, likely due to upregulated IPR

6 heart of huntingtin exon1 fibrils and other polyglutamine aggregates, via measurements of long-range

7 They are effective at seeding polyglutamine aggregation and exhibit cytotoxic effects

8 ward genetic screen in a C. elegans model of polyglutamine aggregation and identified the protein MOA

9 periments and in cell models and accelerated polyglutamine aggregation and toxicity in an oxidation-s

10 ify the driving forces for and mechanisms of polyglutamine aggregation as modulated by N17 and C38.

11 s on the one hand known to markedly increase polyglutamine aggregation rates and on the other hand ha

12 a possible pathway for the initial stages of polyglutamine aggregation, in which beta-hairpin-contain

13 hat control the intrinsic heterogeneities of polyglutamine aggregation.

14 mutant mice, placing a dominant HD knock-in polyglutamine allele onto the slow-aging Snell dwarf gen

15 Internally, the polyglutamine amyloid fibrils are coassembled from diffe

16 er in their flanking domains rather than the polyglutamine amyloid structure.

17 polyglutamine toxicity and prevents purified polyglutamine and Abeta peptides from forming amyloid.

18 Htt17 monomer, as well as the impact of the polyglutamine and proline-rich segments, remains, howeve

19 ormational TR-FRET based immunoassay detects polyglutamine- and temperature-dependent changes on the

20 This suggests a novel mechanism for polyglutamine-associated developmental and cell biologic

21 behavior, huntingtin exon1 fibrils and other polyglutamine-based aggregates contain identical beta-st

22 Polyglutamine-binding protein 1 (PQBP1) is a highly cons

23 Polyglutamine-binding protein 1 (PQBP1) is an RNA-splici

24 These results suggest that polyglutamine can induce an aggregation-promoting activi

25 Polyglutamine-coding (CAG)n repeat expansions in seven d

26 tability of dimers to assess whether a given polyglutamine conformer can be on the aggregation path.

27 We also find that these temperature- and polyglutamine-dependent conformational changes are sensi

28 Overall, our findings identify polyglutamine-dependent inhibition of nucleocytoplasmic

29 on between aberrant accumulation of expanded polyglutamine-dependent insoluble protein species and pa

30 arly, largely independent, manifestations of polyglutamine disease and suggests that additional epige

31 Here, we show that, in a mouse model for the polyglutamine disease dentatorubral-pallidoluysian atrop

32 Consistently, the recovery of lifespan in polyglutamine disease fly models by TERA/VCP/p97 corresp

33 tment exists for the fatal neurodegenerative polyglutamine disease known both as Machado-Joseph disea

34 ate a direct role of arginine methylation in polyglutamine disease pathogenesis.

35 nally blocked versions of one substrate, the polyglutamine disease protein ataxin-3, and showed that

36 protein (VCP)/p97 directly binds to multiple polyglutamine disease proteins (huntingtin, ataxin-1, at

37 contrast to this view, we show that, in the polyglutamine disease spinal and bulbar muscular atrophy

38 ataxia type 2 (SCA2), an autosomal dominant polyglutamine disease.

39 ociation with age at onset when grouping all polyglutamine diseases (HD+SCAs; p = 1.43 x 10(-5) ).

40 etic mechanism modulates age at onset across polyglutamine diseases and could extend to other repeat

41 Polyglutamine diseases are a class of dominantly inherit

42 Polyglutamine diseases are caused by CAG expansions in d

43 Polyglutamine diseases are dominantly inherited neurodeg

44 A key unanswered question in SCA3 and other polyglutamine diseases is the extent to which neurodegen

45 de a novel common pathomechanism in multiple polyglutamine diseases that is mediated by DNA repair fu

46 ng all conditions studied (DM1, DM2, C9-ALS, polyglutamine diseases), reduction of polyglutamine prot

47 The polyglutamine diseases, including Huntington's disease (

48 Polyglutamine diseases, including spinocerebellar ataxia

49 l sclerosis/frontotemporal dementia and with polyglutamine diseases, respectively, localize to neurit

50 orders, such as Alzheimer's, Parkinson's and polyglutamine diseases, share a common pathogenic mechan

51 ults, together with recent findings in other polyglutamine diseases, suggest that CAG repeat expansio

52 RAN translation may also contribute to other polyglutamine diseases.

53 spinocerebellar ataxia type 8, and the nine polyglutamine diseases.

54 structure/function and neurodegeneration in polyglutamine diseases.

55 inal and bulbar muscular atrophy and related polyglutamine diseases.

56 r's disease, Spinomuscular Atrophy and other polyglutamine diseases.

57 sregulation is an important early feature of polyglutamine diseases.

58 enerative disorders, such as Alzheimer's and polyglutamine diseases.

59 n DNA repair genes have wider effects in the polyglutamine diseases.

60 l intervention in SBMA and potentially other polyglutamine diseases.

61 Huntington's disease (HD) is a polyglutamine disorder caused by a CAG expansion in the

62 In a mouse model of the polyglutamine disorder spinocerebellar ataxia type 1 (SC

63 sion of neurodegenerative disease, including polyglutamine disorders such as Huntington's disease and

64 cellular compartment for the pathogenesis of polyglutamine disorders, including Huntington's disease

65 ng to disease pathogenesis in SCA3 and other polyglutamine disorders.

66 ggest a therapeutic potential for LNA-CTG in polyglutamine disorders.

67 a an intramolecular collapse of the expanded polyglutamine domain and discuss the implications of thi

68 Removing the polyglutamine domain of Whi3 restored the pheromone sens

69 sease caused by an abnormal expansion in the polyglutamine encoding CAG repeat of the androgen recept

70 is a neurodegenerative disorder caused by a polyglutamine-encoding CAG repeat expansion in the ATXN3

71 ve disorders that are caused by expansion of polyglutamine-encoding CAG repeats.

72 atrophy (SBMA), a repeat disorder caused by polyglutamine-expanded androgen receptor (polyQ-AR).

73 Expression of polyglutamine-expanded AR causes damage to motor neurons

74 e show that treatment of myotubes expressing polyglutamine-expanded AR with the beta-agonist clenbute

75 found that NLK can phosphorylate the mutant polyglutamine-expanded AR, enhance its aggregation, and

76 R) pathway and decreased the accumulation of polyglutamine-expanded AR.

77 We investigated whether polyglutamine-expanded ATAXIN-1, the protein that underl

78 role in the pathogenic pathways mediated by polyglutamine-expanded ataxin-3 and that phosphorylation

79 with ATXN1, modulates disease phenotypes of polyglutamine-expanded ATXN1 in a Drosophila model of SC

80 cial effects against the toxicity induced by polyglutamine-expanded ATXN1.

81 Here we use a polyglutamine-expanded form of human huntingtin (Htt) wi

82 odies in cells expressing aggregation-prone, polyglutamine-expanded fragments of HTT.

83 t tracking of individual cells enriched with polyglutamine-expanded Htt exon 1 (Httex1) monomers, oli

84 RK) 1/2 activation was not altered by either polyglutamine-expanded Htt or MKP-1.

85 gs suggest new mechanisms for the effects of polyglutamine-expanded HTT.

86 neurons exposed to an N-terminal fragment of polyglutamine-expanded huntingtin (Htt171-82Q), blocking

87 sp70 system in spatial organization of toxic polyglutamine-expanded Huntingtin (Huntingtin with 103Q

88 disorder Huntington's disease (HD), in which polyglutamine-expanded huntingtin (polyQ-htt) is predomi

89 as the capacity to suppress aggregation of a polyglutamine-expanded Huntingtin construct that aggrega

90 s the viability of neuronal cells expressing polyglutamine-expanded huntingtin exon 1 protein fragmen

91 euronal aggregates and inclusions containing polyglutamine-expanded huntingtin protein and peptide fr

92 Overexpression of MKP-1 prevented the polyglutamine-expanded huntingtin-induced activation of

93 2/LIR-3 can also catalyze the aggregation of polyglutamine-expanded huntingtin.

94 rmal Htt and gain of a toxic function by the polyglutamine-expanded mutant Htt protein have been prop

95 tics aimed at correcting the conformation of polyglutamine-expanded proteins as well as the pharmacod

96 Proteolysis of polyglutamine-expanded proteins is thought to be a requi

97 ssociated protein huntingtin with a 103 copy-polyglutamine expansion (HTT gene; htt-103Q).

98 misfolded huntingtin exon I containing a 103-polyglutamine expansion (Htt103QP) as a model substrate

99 or a floxed exon 1 containing the pathogenic polyglutamine expansion (Q97).

100 Although polyglutamine expansion accelerates protein aggregation,

101 In Huntington disease, polyglutamine expansion causes N-terminal huntingtin (Ht

102 Spinocerebellar ataxia type 1 is one of nine polyglutamine expansion diseases and is characterized by

103 n in HD, and may have implications for other polyglutamine expansion diseases in which mutant protein

104 HD is one of nine polyglutamine expansion diseases.

105 disease (HD) is the most commonly inherited polyglutamine expansion disorder, but how mutant Hunting

106 sight, down to the molecular level, into how polyglutamine expansion drives aggregation and explains

107 The clinical threshold of polyglutamine expansion for HD is near 37 repeats, but t

108 Polyglutamine expansion in androgen receptor (AR) is res

109 For example, polyglutamine expansion in ataxin-3 allosterically trigg

110 neurodegenerative disease caused by abnormal polyglutamine expansion in huntingtin (Exp-HTT) leading

111 A polyglutamine expansion in huntingtin (HTT) causes the s

112 rodegenerative disease caused by an abnormal polyglutamine expansion in huntingtin (Htt).

113 untington's disease (HD), which is caused by polyglutamine expansion in huntingtin.

114 Huntington's disease is caused by a polyglutamine expansion in huntingtin.

115 s, we and others have recently reported that polyglutamine expansion in purified or recombinantly exp

116 eurodegenerative disorder caused by abnormal polyglutamine expansion in the amino-terminal end of the

117 Polyglutamine expansion in the androgen receptor (AR) ca

118 progressive neuromuscular disease caused by polyglutamine expansion in the androgen receptor (AR) pr

119 Polyglutamine expansion in the androgen receptor causes

120 Polyglutamine expansion in the androgen receptor, causin

121 fatal neurodegenerative disease caused by a polyglutamine expansion in the coding region of ATXN1.

122 neurodegenerative disease caused by abnormal polyglutamine expansion in the huntingtin protein (Htt).

123 Polyglutamine expansion in the huntingtin protein is the

124 tive disorder caused by an aggregation-prone polyglutamine expansion in the huntingtin protein.

125 erited neurodegenerative disease caused by a polyglutamine expansion in the huntington protein (htt).

126 erited neurodegenerative condition caused by polyglutamine expansion in the N terminus of the hunting

127 th Huntington disease (HD) is triggered by a polyglutamine expansion in the N-terminal region of the

128 se is neurodegenerative disorder caused by a polyglutamine expansion in the N-terminal region of the

129 ataxia type 1 (SCA1), a disease caused by a polyglutamine expansion in the protein ATAXIN1 (ATXN1).

130 Here, we establish that polyglutamine expansion increases the molecular mobility

131 Polyglutamine expansion is a hallmark of nine neurodegen

132 neurodegenerative disorder that results from polyglutamine expansion of the ataxin-7 (ATXN7) protein.

133 Tat-beclin 1 decreases the accumulation of polyglutamine expansion protein aggregates and the repli

134 Polyglutamine expansion within the exon1 of huntingtin l

135 Huntington's disease (HD) is caused by a polyglutamine expansion within the huntingtin (Htt) prot

136 's disease (HD) is caused in large part by a polyglutamine expansion within the huntingtin (Htt) prot

137 a fatal neurodegenerative disease caused by polyglutamine-expansion in huntingtin (HTT).

138 n expression of the highly aggregation-prone polyglutamine-expansion proteins and Abeta-peptide.

139 se (HD) is a neurological disorder caused by polyglutamine expansions in mutated Huntingtin (mHtt) pr

140 ominant neurodegenerative disorder caused by polyglutamine expansions in the amino-terminal region of

141 cts males, results from a CAG triplet repeat/polyglutamine expansions in the androgen receptor (AR) g

142 in yeast and flies, and intermediate-length polyglutamine expansions in the ataxin-2 gene increase r

143 an indirect and poorly understood manner by polyglutamine expansions in the huntingtin (HTT) protein

144 the huntingtin protein (mHTT) with aberrant polyglutamine expansions.

145 dence for beta-arch-containing structures in polyglutamine fibrils and open future possibilities for

146 s the driving force, vis-a-vis homopolymeric polyglutamine, for forming insoluble aggregates.

147 Homopolymeric polyglutamine forms a mixture of amorphous aggregates an

148 neurodegenerative disease-related proteins (polyglutamine, huntingtin, ataxin-1, and superoxide dism

149 Abeta aggregation mechanism that uses Abeta-polyglutamine hybrid peptides designed to retard amyloid

150 Huntingtin-linked polyglutamine initially accumulates in nuclei, leading t

151 mice, accumulation of RanGAP1 together with polyglutamine is shifted to perinuclear and cytoplasmic

152 that although the alpha-helical conformer of polyglutamine is very stable, dimers of alpha-helices la

153 t onset of disease decreases with increasing polyglutamine length in these proteins and the normal le

154 ino acids 171, 463, 536, 552, and 586 with a polyglutamine length of 148.

155 1 aggregation in cells with respect to time, polyglutamine length, expression levels, cell survival,

156 d that HDAC4 associates with huntingtin in a polyglutamine-length-dependent manner and co-localises w

157 We identified two variable polyglutamine microsatellites in chimpanzees and orangut

158 In the absence of polyglutamine, MOAG-2/LIR-3 regulates the RNA polymerase

159 ay impair FOXO protective activity in mutant polyglutamine neurons, suggesting that neurons are unabl

160 The similarity to polyglutamine nucleation suggests that monomeric nuclei

161 We report that synthetic polyglutamine oligomers and cerebrospinal fluid (CSF) fr

162 l fragment delays aggregation onset by Abeta-polyglutamine peptides and redirects assembly of Abeta42

163 nhibit the formation of amyloid fibrils from polyglutamine peptides associated with neurodegenerative

164 ogy calls for understanding the structure of polyglutamine peptides in the early stages of aggregatio

165 to simultaneously probe fibril formation in polyglutamine peptides, the aggregating subunit associat

166 ington disease (HD) is caused by an expanded polyglutamine (poly(Q)) repeat near the N terminus of th

167 otein (mHTT) with an expanded amino-terminal polyglutamine (poly(Q)) stretch.

168 egenerative disease caused by expansion of a polyglutamine [poly(Q)] tract in ATXN7, a subunit of the

169 Polyglutamine (polyQ) amyloid fibrils are observed in di

170 AT yeast homologs, NMA1 and NMA2, suppresses polyglutamine (PolyQ) and alpha-synuclein-induced cytoto

171 ubiquitinated inclusions immunoreactive for polyglutamine (polyQ) antibodies.

172 Huntington's disease (HD) is a polyglutamine (polyQ) disease caused by aberrant expansi

173 further show that PML deficiency exacerbates polyglutamine (polyQ) disease in a mouse model of spinoc

174 odegenerative disorder caused by an expanded polyglutamine (polyQ) domain near the N-terminus of the

175 ington's disease is caused by expansion of a polyglutamine (polyQ) domain within exon 1 of the huntin

176 ith an increased aggregation propensity of a polyglutamine (polyQ) expansion in exon 1 of mutant hunt

177 It is caused by a polyglutamine (polyQ) expansion in the androgen receptor

178 rebellar ataxia type 7 (SCA7) is caused by a polyglutamine (polyQ) expansion in the ataxin-7 protein,

179 -onset neurodegenerative disease caused by a polyglutamine (polyQ) expansion in the N-terminal region

180 Polyglutamine (polyQ) expansion of the androgen receptor

181 n an encoded region of the gene resulting in polyglutamine (polyQ) expansion which has been assumed t

182 A7) is a neurodegenerative disease caused by polyglutamine (polyQ) expansion within the N-terminal re

183 nerative disorder caused by the expansion of Polyglutamine (polyQ) in exon 1 of the Huntingtin protei

184 Polyglutamine (polyQ) peptides are a useful model system

185 the toxic molecular species in the expanded polyglutamine (polyQ) repeat diseases range from various

186 neurodegenerative disorder caused by a CAG - polyglutamine (polyQ) repeat expansion in the ataxin-7 g

187 Polyglutamine (polyQ) repeat expansion in the deubiquiti

188 SBMA is caused by CAG-polyglutamine (polyQ) repeat expansions in the androgen

189 ington's disease is caused by expansion of a polyglutamine (polyQ) repeat in the huntingtin protein.

190 odegenerative disorder caused by an expanded polyglutamine (polyQ) repeat in the TATA-box-binding pro

191 odegenerative disorder caused by an expanded polyglutamine (polyQ) repeat within the protein huntingt

192 Aggregates of proteins containing polyglutamine (polyQ) repeats are strongly associated wi

193 esulting mutant protein (mHtt) with extended polyglutamine (polyQ) sequence at the N terminus leads t

194 rongly dependent on the repeat length of the polyglutamine (polyQ) sequence in the disease protein.

195 Polyglutamine (polyQ) sequences are found in a variety o

196 The conformational preferences of polyglutamine (polyQ) sequences are of major interest be

197 of huntingtin protein arising from expanded polyglutamine (polyQ) sequences in the exon-1 region of

198 generative disorder caused by expansion of a polyglutamine (polyQ) stretch within the Huntingtin (Htt

199 sease caused by an abnormal expansion in the polyglutamine (polyQ) track of the Huntingtin (HTT) prot

200 are enhanced in the presence of the expanded polyglutamine (polyQ) tract and are stronger in the nucl

201 Fragments of proteins containing an expanded polyglutamine (polyQ) tract are thought to initiate aggr

202 gene, which is translated into an elongated polyglutamine (polyQ) tract in AR protein (ARpolyQ).

203 h is caused by a pathological expansion of a polyglutamine (polyQ) tract in the coding region of the

204 gton's disease (HD) is caused by an expanded polyglutamine (polyQ) tract in the huntingtin (htt) prot

205 ng catalytic activity or bearing an expanded polyglutamine (polyQ) tract led to partially overlapping

206 is unusual in that it includes a C-terminal polyglutamine (polyQ) tract that is absent in nonrodent

207 alpha1ACT also contains the polyglutamine (polyQ) tract that, when expanded, causes

208 Expansion of the polyglutamine (polyQ) tract within the androgen receptor

209 of huntingtin (HTT) fragments with expanded polyglutamine (polyQ) tracts are a pathological hallmark

210 genes, TR copy number mutations that expand polyglutamine (polyQ) tracts beyond a certain threshold

211 Expansions of preexisting polyglutamine (polyQ) tracts in at least nine different

212 degenerative diseases are caused by expanded polyglutamine (polyQ) tracts in different proteins, such

213 Expansions of polyglutamine (polyQ) tracts in nine different proteins

214 The aggregation of proteins with expanded polyglutamine (polyQ) tracts is directly relevant to the

215 caused by cell death after the expansion of polyglutamine (polyQ) tracts longer than approximately 4

216 zed by aggregation of proteins with expanded polyglutamine (polyQ) tracts.

217 The aggregation of polyglutamine (polyQ)-containing proteins is at the orig

218 a type 6 (SCA6) belongs to the family of CAG/polyglutamine (polyQ)-dependent neurodegenerative disord

219 xpansion of CAG repeats encoding consecutive polyglutamines (polyQ) in the corresponding disease prot

220 ists strong correlation between the extended polyglutamines (polyQ) within exon-1 of Huntingtin prote

221 Changes caused by an expanded polyglutamine protein are possibly influenced by endogen

222 ll-length protein, challenging the notion of polyglutamine protein fragment-associated toxicity by re

223 elated decline in chaperone activity affects polyglutamine protein function that is important for the

224 9-ALS, polyglutamine diseases), reduction of polyglutamine protein products, relocalization of repeat

225 y and involved in the clearance of misfolded polyglutamine protein, is strongly recruited to the muta

226 oratory and wild strains and disease-related polyglutamine proteins expressed in both yeast and mamma

227 Mutant polyglutamine proteins impair accumulation of TERA/VCP/p

228 Although normal and mutant polyglutamine proteins interact with TERA/VCP/p97, only

229 The accepted view for many polyglutamine proteins is that proteolysis of the mutant

230 cell lines that expressed aggregation-prone polyglutamine proteins over several months.

231 f the aggregation pathway for toxic expanded polyglutamine proteins.

232 he expression of TDP-43, alpha-synuclein, or polyglutamine proteins.

233 The polyglutamine-proximal portions of these domains are imm

234 diseases, namely at early stages of another polyglutamine-related disorder such as Huntington's dise

235 Polyglutamine repeat expansion in ataxin-3 causes neurod

236 at residue T3) of a protein associated with polyglutamine repeat expansion, namely Huntingtin, and c

237 neurodegenerative disorder, caused by a CAG/polyglutamine repeat expansion, which is associated with

238 ative disorder caused by an expansion of the polyglutamine repeat in the first exon in the androgen r

239 odegenerative disorder caused by an extended polyglutamine repeat in the N terminus of the Huntingtin

240 This DNA sequence translates to a polyglutamine repeat in the protein product, leading to

241 nal properties in a manner dependent on both polyglutamine repeat length and temperature but independ

242 ng the aggregation free energy profile for a polyglutamine repeat with site-specific PG mutations tha

243 ng a mutant huntingtin protein (mHTT) with a polyglutamine-repeat expansion.

244 cular atrophy mice that carry 100 pathogenic polyglutamine repeats in the androgen receptor, and deve

245 degenerative disease, caused by expansion of polyglutamine repeats in the Huntingtin gene, with longe

246 is a rare genetic disease caused by expanded polyglutamine repeats in the huntingtin protein resultin

247 disorder caused by an abnormal expansion of polyglutamine repeats in the N-terminal of huntingtin.

248 HD is triggered by an expansion of polyglutamine repeats in the protein huntingtin (Htt), i

249 ncoding huntingtin (Htt) leading to expanded polyglutamine repeats of mutant Htt (mHtt) that elicit o

250 pendent cohort of 1,462 subjects with HD and polyglutamine SCAs, and genotyped single-nucleotide poly

251 relevant to pathogenic amyloid formation by polyglutamine segments in human proteins.

252 taxin-1, ataxin-7 and androgen receptor) via polyglutamine sequence.

253 A stochastic polyglutamine-specific aggregation mechanism is introduc

254 The protein ataxin-3 carries a polyglutamine stretch close to the C-terminus that trigg

255 CAG repeat expansion leading to an elongated polyglutamine stretch in huntingtin.

256 cleotide CAG repeat expansion that encodes a polyglutamine stretch in the huntingtin (HTT) protein.

257 of an RNA-binding protein, and deletion of a polyglutamine stretch in this protein results in random

258 on's disease (HD) results from expansions of polyglutamine stretches (polyQ) in the huntingtin protei

259 re we find DnaJB6-protected yeast cells from polyglutamine toxicity and cured yeast of both [URE3] pr

260 efects cause myopathies, protects cells from polyglutamine toxicity and prevents purified polyglutami

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

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

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

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

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

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

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

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

269 1 (SCA1), a disease caused by expansion of a polyglutamine tract in ataxin 1 (ATXN1).

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

290 n requires expressing ATXN1 with an expanded polyglutamine tract.

291 1-17 domains and possibly with the proximal polyglutamine tract.

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

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

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

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

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

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

298 o suppress aggregation of proteins with long polyglutamine tracts.

299 This regulation is lost in the presence of polyglutamine, which mislocalizes MOAG-2/LIR-3 from the

300 We designed polyglutamines with a few lysines inserted to overcome t