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 n-3 pathogenicity is saliently controlled by polyglutamine-adjacent ubiquitin-interacting motifs (UIM

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

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

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 her, both ER hormesis and DR protect against polyglutamine aggregation in an IRE-1-dependent manner.

11 ted genetic screen for germline modifiers of polyglutamine aggregation in muscle cells.

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

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

14 Internally, the polyglutamine amyloid fibrils are coassembled from diffe

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

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

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

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

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

20 ent, advances in preclinical studies for the polyglutamine ataxias and the initial clinical applicati

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

22 lencing of the AR-RGN top key driver, PQBP1 (polyglutamine binding protein 1), significantly curbed c

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 aggregation and concomitant toxicity of the polyglutamine-containing N-terminal region of the huntin

28 ve cells, we show here that an amyloidogenic polyglutamine-containing protein first forms small, amor

29 estores tRNA(Gln) charging and the levels of polyglutamine-containing proteins.

30 nes, which are generally thought to suppress polyglutamine degeneration.

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

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

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

34 ms an amyloid core resembling those of other polyglutamine deposits.

35 iously observed in a Drosophila model of the polyglutamine disease Dentatorubral-pallidoluysian atrop

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

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

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

39 This heterogeneity may also extend to how polyglutamine disease proteins are handled by cellular p

40 ial differential regulation by UBQLN2 of two polyglutamine disease proteins, huntingtin (HTT) and ata

41 s reveal a selective action of UBQLN2 toward polyglutamine disease proteins, indicating that polyglut

42 (UBQLN2) selectively interacts with specific polyglutamine disease proteins.

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

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

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

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

47 Polyglutamine diseases are a class of dominantly inherit

48 Polyglutamine diseases are caused by CAG expansions in d

49 Polyglutamine diseases are dominantly inherited neurodeg

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

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

52 es-Alzheimer's disease, Parkinson's disease, polyglutamine diseases, and amyotrophic lateral sclerosi

53 The polyglutamine diseases, including Huntington's disease (

54 Polyglutamine diseases, including spinocerebellar ataxia

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

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

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

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

59 l intervention in SBMA and potentially other polyglutamine diseases.

60 RAN translation may also contribute to other polyglutamine diseases.

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

62 structure/function and neurodegeneration in polyglutamine diseases.

63 inal and bulbar muscular atrophy and related polyglutamine diseases.

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

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

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

67 Although caused by a similar mutation, polyglutamine disorders are distinct, implicating non-po

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

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

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

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

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

73 enerative disease caused by expansion of the polyglutamine domain in the first exon of huntingtin (Ht

74 Although the polyglutamine domain is important for fibril formation,

75 Removing the polyglutamine domain of Whi3 restored the pheromone sens

76 This expansion results in an elongated polyglutamine domain that increases the propensity of hu

77 To determine the role of ataxin-3's non-polyglutamine domains in disease, we utilized a new, all

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

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

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

81 he activation of GCN2 and the translation of polyglutamine-encoding transcripts serve as key sensors

82 as Ag source was directly demonstrated using polyglutamine-equipped model substrates.

83 artificial chromosome mouse model expressing polyglutamine expanded ATXN2.

84 Here we evaluate mutant polyglutamine-expanded (mHTT) and polyglutamine-independ

85 We show that, in the presence of polyglutamine-expanded (polyQ-expanded) huntingtin (HTT)

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

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

88 drogen-dependent nuclear accumulation of the polyglutamine-expanded AR is an essential step in the pa

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

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

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

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

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

94 Proteolytic fragmentation of polyglutamine-expanded ataxin-3 is a concomitant and mod

95 a functional interaction between normal and polyglutamine-expanded ATXN3 allelic variants.

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

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

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

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

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

101 2/LIR-3 can also catalyze the aggregation of polyglutamine-expanded huntingtin.

102 In SCA1 mice, polyglutamine-expanded mutant ataxin-1 led to the increa

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

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

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

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

107 Although polyglutamine expansion accelerates protein aggregation,

108 yglutamine disease proteins, indicating that polyglutamine expansion alone is insufficient to promote

109 chromatin remodeling complex, is subject to polyglutamine expansion at the amino terminus, causing s

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

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

112 Divergent protein context helps explain why polyglutamine expansion diseases differ clinically and p

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

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

115 Polyglutamine expansion in androgen receptor (AR) is res

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

117 SCA3 is caused by polyglutamine expansion in ataxin-3.

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

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

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

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

122 Polyglutamine expansion in proteins can cause selective

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

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

125 Polyglutamine expansion in the androgen receptor (AR) ca

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

127 Polyglutamine expansion in the androgen receptor causes

128 Polyglutamine expansion in the androgen receptor, causin

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

130 Polyglutamine expansion in the huntingtin protein is the

131 on in exon 1 of the HTT gene, resulting in a polyglutamine expansion in the huntingtin protein.

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

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

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

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

136 Polyglutamine expansion is a hallmark of nine neurodegen

137 Polyglutamine expansion results in an aggregation-prone

138 Polyglutamine expansion within the exon1 of huntingtin l

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

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

141 Polyglutamine expansion within the N-terminal region of

142 s, and inclusion bodies caused by huntingtin polyglutamine expansion.

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

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

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

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

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

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

149 Polyglutamine expansions in the transcriptional co-repre

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

151 n of 103Q-GFP, a foreign protein with a long polyglutamine extension, resulted in distribution of thi

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

153 luding concentration and the role of the non-polyglutamine flanking domains.

154 acilitated by the C-terminal tail, while the polyglutamine forms an amyloid core resembling those of

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

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

157 ate mutant polyglutamine-expanded (mHTT) and polyglutamine-independent HTT specific immunoassays for

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

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

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

161 r AOO (average of 25 years) despite the same polyglutamine length as in individuals with the interrup

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

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

164 or to AOO of HD and is more significant than polyglutamine length, which is not altered in these indi

165 We identified two variable polyglutamine microsatellites in chimpanzees and orangut

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

167 taxia type 3 (SCA3) belongs to the family of polyglutamine neurodegenerations.

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

169 The similarity to polyglutamine nucleation suggests that monomeric nuclei

170 d by an expansion mutation of the N-terminal polyglutamine of huntingtin (mHTT).

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

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

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

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

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

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

177 Polyglutamine (polyQ) amyloid fibrils are observed in di

178 ubiquitinated inclusions immunoreactive for polyglutamine (polyQ) antibodies.

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

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

181 Polyglutamine (polyQ) disorders are a group of nine neur

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

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

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

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

186 (SBMA) is a neuromuscular disease caused by polyglutamine (polyQ) expansion in the androgen receptor

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

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

189 nocerebellar ataxias 17 (SCA17) is caused by polyglutamine (polyQ) expansion in the TATA box-binding

190 Polyglutamine (polyQ) expansion of the androgen receptor

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

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

193 Polyglutamine (polyQ) peptides are a useful model system

194 ucturally diverse model substrates including polyglutamine (PolyQ) proteins, firefly luciferase, and

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

196 Polyglutamine (polyQ) repeat expansion in the deubiquiti

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

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

199 ELF3 contains a polyglutamine (polyQ) repeat(8-10), embedded within a pr

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

201 Furthermore, polyglutamine (polyQ) repeats form toxic aggregates in s

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

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

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

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

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

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

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

209 ntingtin protein (mHTT) contains an expanded polyglutamine (polyQ) tract and causes Huntington's dise

210 thogenic HD CAG-expansion mutations create a polyglutamine (polyQ) tract at the N terminus of HTT tha

211 The expanded polyglutamine (polyQ) tract form of ataxin-1 drives dise

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

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

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

215 T) protein carrying the elongated N-terminal polyglutamine (polyQ) tract misfolds and forms protein a

216 Polyglutamine (polyQ) tract polymorphism within the huma

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

218 sed methods use Abs that target the expanded polyglutamine (polyQ) tract to quantify mutant huntingti

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

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

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

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

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

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

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

226 tingtin (HTT) fragments with variable length polyglutamine (polyQ) tracts.

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

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

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

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

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

232 Ataxin-1 (ATXN1) is a ubiquitous polyglutamine protein expressed primarily in the nucleus

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

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

235 9-ALS, polyglutamine diseases), reduction of polyglutamine protein products, relocalization of repeat

236 llar ataxia type 1 (SCA1) caused by an ATXN1 polyglutamine protein, although subtle variations in WT

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

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

239 Hsc70-4 also enhances pathogenicity of other polyglutamine proteins.

240 The polyglutamine-proximal portions of these domains are imm

241 comprises an amphiphilic domain (htt(NT)), a polyglutamine (Q (n) ) tract, and a proline-rich sequenc

242 isorder that is caused by the expansion of a polyglutamine region within the huntingtin (HTT) protein

243 ted in size by the stretching entropy of the polyglutamine region.

244 mine disorders are distinct, implicating non-polyglutamine regions of disease proteins as regulators

245 lar ataxia type 7 (SCA7) is an inherited CAG-polyglutamine repeat disorder.

246 Polyglutamine repeat expansion in ataxin-3 causes neurod

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

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

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

250 al level, we found that the expansion of the polyglutamine repeat leads to a stabilization of ataxin-

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

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

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

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

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

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

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

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

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

260 of a cysteine-adenine-guanine trinucleotide (polyglutamine) repeats in exon one of the human huntingt

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

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

263 A stochastic polyglutamine-specific aggregation mechanism is introduc

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

296 o suppress aggregation of proteins with long polyglutamine tracts.

297 ntingtin proteins containing short and large polyglutamine tracts.

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

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

300 hat the energetics of cross-beta stacking by polyglutamine would produce fibrils with many alignment