ライフサイエンスコーパス: CAG repeat

1 do-Joseph Disease, also contains an expanded CAG repeat.

2 ulnerable to the dominant effects of the HTT CAG repeat.

3 et are determined largely by the size of HTT CAG repeat.

4 of the huntingtin protein containing a long CAG repeat.

5 diseases caused by expansion of a translated CAG repeat.

6 were inversely correlated with the number of CAG repeats.

7 lutamine (polyQ) domains encoded by expanded CAG repeats.

8 hat prevents expansion of disease-associated CAG repeats.

9 aused by expansion of polyglutamine-encoding CAG repeats.

10 iate the stress-induced mutagenesis (SIM) of CAG repeats.

11 ntingtin gene (mHTT), which harbors expanded CAG repeats.

12 omatic instability of highly expanded (CTG)*(CAG) repeats.

13 are caused by unstable expansions of (CTG)*(CAG) repeats.

14 The ORs were 2.70 (95% CI, 1.47-4.93) for 31 CAG repeats, 11.09 (95% CI, 4.16-29.57) for 32 repeats,

15 untingtin alleles and other mRNAs containing CAG repeats [14].

16 sporadic +1 frameshift to generate from the CAG repeat a trans-frame AGC repeat-encoded product.

17 RNA duplexes; (ii) the sequences surrounding CAG repeats affect allele-selectivity of anti-CAG oligon

18 ich contains the human HD mutation with a 51 CAG repeat allele, exhibits motor deficits that begin wh

19 is a neurodegenerative disorder caused by a CAG repeat amplification in the gene huntingtin (HTT) th

20 at HD transgenic mice model (R6/2) (with 144 CAG repeat and exon 1) during late-stage pathology, had

21 ipts dependent on the length of the targeted CAG repeat and on the CTG repeat length and concentratio

22 the intergenerational instability of the HD CAG repeat and the striatal-specific somatic HD CAG repe

23 uences associated with Huntington's disease (CAG repeats) and myotonic dystrophy type 1 (CTG repeats)

24 t length and genetic background (115 and 250 CAG repeats, and a mixed CBAxC57 or pure C57 background)

25 hanges, or cell loss in the tgHD rat with 51 CAG repeats, and suggest that this protocol could provid

26 In budding yeast, we found that expanded CAG repeats are more likely than unexpanded repeats to l

27 Expanded CAG repeats are prone to breakage, and repair of the bre

28 which phenotypes associated with expanded HD CAG repeats are studied.

29 , these perturbations are overcome in longer CAG repeats, as demonstrated by studies of isolated and

30 , relevant to myotonic dystrophy type I, and CAG repeats associated with poly-glutamine diseases.

31 llite, most commonly encoding (as in mice) a CAG repeat-associated glutamine-rich domain.

32 mice promoted intergenerational expansion of CAG repeats at the murine spinocerebellar ataxia type 1

33 persistent double-stranded breaks, expanded CAG repeats at the nuclear envelope associate with pores

34 ops can trigger repeat instability at (CTG).(CAG) repeats, but the mechanism of this is unclear.

35 For example, expanded CAG repeats can cause Huntington's and other disease thr

36 specific expansion of polyglutamine-encoding CAG repeats can cause neurodegenerative disorders, inclu

37 nucleic acid antisense oligomers that target CAG repeats can preferentially inhibit mutant ataxin-3 a

38 cally alters the Fnu4HI digestion pattern of CAG repeat chromatin.

39 rroneously harbors a tandem duplicate of the CAG repeat-containing exon, and a corrected model, intro

40 sion repair (BER) is responsible for causing CAG repeat contractions downstream of Fcy1, but not frag

41 A significant increase in CAG repeat contractions was also observed, consistent wi

42 (SSBR) in modulating transcription-dependent CAG repeat contractions.

43 ine, with and without adjustment for age and CAG repeat count.

44 ned significant after adjustment for age and CAG repeat count.

45 of a DNA base lesion can also contribute to CAG repeat deletions that were initiated by the formatio

46 e-onset HD (JHD) lines, which appeared to be CAG repeat-dependent and mediated by the loss of signali

47 There are now 10 expanded CAG repeat diseases in which both disease risk and age o

48 esponsible for neurodegeneration in expanded CAG repeat diseases such as Huntington's disease.

49 of their central importance in the expanded CAG repeat diseases that include Huntington's disease.

50 the nature of the toxic species in expanded CAG repeat diseases.

51 other neurodegenerative diseases, including CAG repeat disorders, or in peripheral tissues of c9FTD/

52 clusions and related neuropathologies of the CAG-repeat disorders are linked to the expansion of a po

53 We show further that SIM of CAG repeats does not involve mismatch repair, nucleotide

54 ere, we discovered size-limited expansion of CAG repeats during repair of 8-oxoG in a wild-type mouse

55 sions, and H4K16 acetylation was enriched at CAG repeats during S phase.

56 disease is initiated by the expression of a CAG repeat-encoded polyglutamine region in full-length h

57 erative disease caused by the expansion of a CAG repeat encoding a polyglutamine tract in Ataxin-1 (A

58 egenerative disease caused by expansion of a CAG repeat encoding a polyglutamine tract in ATXN7, a co

59 ative disorders caused by the expansion of a CAG repeat encoding glutamine within the coding region o

60 egenerative disorders caused by expansion of CAG repeats encoding a glutamine tract in the disease-ca

61 isorders strongly depend on the expansion of CAG repeats encoding consecutive polyglutamines (polyQ)

62 egenerative disorders caused by expansion of CAG repeats encoding polyglutamine (polyQ) tracts in CAC

63 enerative diseases are due to expansion of a CAG repeat- encoding glutamine within the open reading f

64 mortality, so we tested whether the expanded CAG repeat exerts a dominant influence on age at death a

65 xoguanine (8-oxoG) is implicated in neuronal CAG repeat expansion associated with Huntington disease,

66 nocopy of Huntington's disease caused by CTG/CAG repeat expansion at the Junctophilin-3 (JPH3) locus.

67 is the most prevalent member of a family of CAG repeat expansion disorders.

68 fatal neurodegenerative disorder caused by a CAG repeat expansion encoding a polyglutamine tract in t

69 ATXN2, ATXN3, ATXN7, TBP and CACNA1A and the CAG repeat expansion gene PPP2R2B.

70 ssive neurodegenerative disorder caused by a CAG repeat expansion in exon 1 of huntingtin (HTT).

71 It is caused by a CAG repeat expansion in exon 1 of the HTT gene that tran

72 HD) is determined largely by the length of a CAG repeat expansion in HTT but is also influenced by ot

73 ninety-eight patients carried a pathological CAG repeat expansion in HTT, whereas 28 patients (12 wom

74 an X-linked motor neuron disease caused by a CAG repeat expansion in the androgen receptor gene.

75 polyglutamine expansion disorder caused by a CAG repeat expansion in the AR gene.

76 dominant neurodegenerative disease caused by CAG repeat expansion in the ATXN2 gene.

77 disorder caused by a polyglutamine-encoding CAG repeat expansion in the ATXN3 gene.

78 Huntington's disease (HD), caused by a CAG repeat expansion in the huntingtin (HTT) gene, is ch

79 HD is caused by a CAG repeat expansion in the Huntingtin (HTT) gene, trans

80 HD is caused by a CAG repeat expansion in the huntingtin (HTT) gene, while

81 Huntington's disease (HD) is caused by a CAG repeat expansion in the huntingtin (HTT) gene.

82 Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT.

83 fatal neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin gene.

84 rited neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin gene.

85 The CAG repeat expansion in the Huntington's disease gene HT

86 inocerebellar ataxia 12 (SCA12) is caused by CAG repeat expansion in the non-coding region of the PPP

87 ion of this method for introduction of a 162 CAG repeat expansion into the hAR 254kb BAC is shown.

88 rited neurodegenerative disorder caused by a CAG repeat expansion leading to an elongated polyglutami

89 ase (HD) reflects dominant consequences of a CAG repeat expansion mutation in HTT.

90 of nuclear mutant huntingtin and somatic HD CAG repeat expansion predict that the initiation of each

91 HD is caused by a trinucleotide CAG repeat expansion that encodes a polyglutamine stretc

92 The CACNA1A CAG repeat expansion was excluded.

93 ssive neurodegenerative disorder caused by a CAG repeat expansion within exon 1 of HTT, encoding hunt

94 odegenerative disorder caused by an abnormal CAG repeat expansion within exon 1 of the huntingtin gen

95 repeat and the striatal-specific somatic HD CAG repeat expansion, nuclear mutant huntingtin accumula

96 he protein-coding sequence evolved through a CAG repeat expansion.

97 HDL2 is caused by a CTG/CAG repeat expansion.

98 oordination during long-patch BER results in CAG repeat expansion.

99 (HD) reflects the dominant consequences of a CAG-repeat expansion in HTT.

100 mine (polyQ) diseases, which are caused by a CAG-repeat expansion within the coding region of the ass

101 However, the prevalence of HTT CAG repeat expansions among individuals diagnosed with m

102 es, which are believed to be responsible for CAG repeat expansions associated with certain human neur

103 Msh3-/- cells are severely defective for CTG*CAG repeat expansions but show full activity on contract

104 Recent studies have demonstrated that CAG repeat expansions can be initiated by oxidative DNA

105 n other polyglutamine diseases, suggest that CAG repeat expansions can promote aberrant splicing to p

106 CTG*CAG repeat expansions cause at least twelve inherited ne

107 Huntington disease phenocopies without CAG repeat expansions in HTT are not rare, occurring in

108 SBMA is caused by CAG repeat expansions in the androgen receptor (AR) gene

109 a fatal neurodegenerative disease caused by CAG repeat expansions in the gene encoding huntingtin (H

110 Huntington's disease is caused by CAG repeat expansions in the HTT gene, which encodes the

111 Intermediate or full CAG repeat expansions of ATXN2 are associated with ALS.

112 nd her uncle with ALS have full pathological CAG repeat expansions of ATXN2.

113 rebellar ataxia type 3 (SCA3), are caused by CAG repeat expansions that encode abnormally long glutam

114 tion were required to prevent Rad5-dependent CAG repeat expansions, and H4K16 acetylation was enriche

115 age by flap endonuclease 1 (FEN1) to mediate CAG repeat expansions.

116 CAG repeats form stable hairpin structures, which are be

117 ase Fcy1 significantly decreased the rate of CAG repeat fragility and contractions in the rnh1Deltarn

118 Nup84 pore subcomplex and Slx5/8 suppresses CAG repeat fragility and instability.

119 Shorter wild-type alleles, other genomic CAG-repeat genes, and neighboring genes were unaffected.

120 base lesion located in the loop region of a CAG repeat hairpin can remove the hairpin, attenuating r

121 nt, the widely used mutant huntingtin-exon 1 CAG repeat HD transgenic mice model (R6/2) (with 144 CAG

122 es mouse HD gene homolog (Hdh) with extended CAG repeat- HdhQ250, which was derived from the selectiv

123 electively target sequences flanking the HTT CAG repeat (HTTex1a and HTTex1b).

124 th expanded alleles containing 44, 77 or 109 CAG repeats, HTTex1a and HTTex1b were effective in suppr

125 atient tissue was not apparent in the mutant CAG repeat huntingtin full-length HD (YAC72) transgenic

126 ion of huntingtin (Htt) exon 1 with expanded CAG repeats, implicated in Huntington pathology, undergo

127 disease caused by expansion of a translated CAG repeat in Ataxin-1 (ATXN1).

128 ype 1 is caused by expansion of a translated CAG repeat in ataxin1 (ATXN1).

129 nerational and somatic instability of the HD CAG repeat in C57BL/6 and FVB/N backgrounds compared wit

130 rodegenerative disease caused by an expanded CAG repeat in HTT.

131 d up to a 15-fold increase in changes to the CAG repeat in human and rodent cell lines, and that long

132 (MND) caused by an abnormal expansion of the CAG repeat in the androgen receptor (AR) gene on the X-c

133 untington's disease is caused by an expanded CAG repeat in the gene encoding huntingtin (HTT), result

134 odegenerative disorder caused by an expanded CAG repeat in the gene encoding huntingtin (HTT).

135 ombination at an ectopic hairpin forming CTG/CAG repeat in the HeLa genome.

136 monogenic disease, is caused by an expanded CAG repeat in the HTT gene exceeding 35 units.

137 erative disease caused by the expansion of a CAG repeat in the HTT gene.

138 generative disorder caused by expansion of a CAG repeat in the huntingtin (Htt) gene.

139 gton's disease (HD) is caused by an expanded CAG repeat in the Huntingtin (HTT) gene.

140 It is caused by expansion of the CAG repeat in the huntingtin gene (HTT) and characterize

141 riably fatal, HD is caused by expansion of a CAG repeat in the Huntingtin gene, creating an extended

142 odegenerative disorder caused by an expanded CAG repeat in the huntingtin gene, which encodes an abno

143 odegenerative disorder caused by an expanded CAG repeat in the huntingtin gene.

144 disorder caused by expansion of a translated CAG repeat in the N terminus of the huntingtin (htt) pro

145 Although expansion of CAG repeats in ATAXIN1 (ATXN1) causes Spinocerebellar at

146 iation with the longer of the two allelic HD CAG repeats in both the non-HD and HD ranges.

147 nt for characterizing the effects of DSBs on CAG repeats in cells.

148 uclease HI stimulates the instability of CTG.CAG repeats in E. coli.

149 ributing to intergenerational instability of CAG repeats in mice and in humans.

150 e disease is caused by abnormal expansion of CAG repeats in the gene encoding huntingtin, but how mut

151 ve disorder caused by an increased number of CAG repeats in the HTT gene coding for huntingtin.

152 eurodegenerative disorder caused by expanded CAG repeats in the huntingtin (HTT) gene.

153 D) is caused by a pathological elongation of CAG repeats in the huntingtin protein gene and is charac

154 We first determined that the ZFNs cleave CAG repeats in vitro.

155 fferent numbers of cytosine-adenine-guanine (CAG) repeats in a fragment of the gene responsible for H

156 showed previously that transcription through CAG repeats induces their instability.

157 which have human huntingtin exon 1 with 140 CAG repeats inserted into the endogenous mouse huntingti

158 f pathways involved in transcription-induced CAG repeat instability and begin to define their interre

159 netic modifiers of both intergenerational HD CAG repeat instability and striatal-specific phenotypes.

160 suggests that tissue-to-tissue variation in CAG repeat instability arises, in part, by different und

161 erns of CpG methylation, plays a key role in CAG repeat instability in human cells and in the male an

162 also showed that transcription-dependent CTG.CAG repeat instability in human cells is stimulated by s

163 as to whether or not MutSbeta is involved in CAG repeat instability in humans.

164 e germline; however, it dramatically reduces CAG repeat instability in neuronal tissues-striatum, hip

165 NOT2/3/5 conserved domain, as a modifier of CAG repeat instability in vivo.

166 tentative pathway for transcription-induced CAG repeat instability that can account for the contract

167 e culture assay for identifying modifiers of CAG repeat instability, we found that transfection of ZF

168 ved in the pathway for transcription-induced CAG repeat instability.

169 loop formation and reveal two mechanisms for CAG repeat instability: one mediated by cytosine deamina

170 a type 3 recapitulates key features of human CAG-repeat instability, including large repeat changes a

171 dary structure-forming DNA sequences such as CAG repeats interfere with replication and repair, provo

172 The length of the CAG repeat is inversely correlated with age of onset (AO

173 ypothesis that somatic instability of the HD CAG repeat is itself a modifier of disease.

174 that a cis-regulatory effect of the expanded CAG repeat is not a critical component of the underlying

175 ich the intron immediately downstream of the CAG repeat is retained.

176 The HD CAG repeat is somatically unstable, undergoing progressi

177 ssion of antisense transcripts with expanded CAG repeats is limited.

178 nsion beyond a threshold of approximately 35 CAG repeats is the cause of several human diseases.

179 n of 1 x 1 nucleotide AA internal loops in r(CAG) repeats is anti-anti but can adopt syn-anti dependi

180 Expanded CAG repeats lead to debilitating neurodegenerative disor

181 isease--huntingtin--results from an expanded CAG repeat leading to a polyglutamine strand of variable

182 icipants aged 26 to 57 years had an expanded CAG repeat length (>/= 37).

183 ease can be improved beyond that obtained by CAG repeat length and age alone.

184 ith those who did not, after controlling for CAG repeat length and age-related risk (p=0.006 and 0.00

185 sease progression and the combined effect of CAG repeat length and age.

186 ificant predictors of motor diagnosis beyond CAG repeat length and age.

187 G repeat length, age, and the interaction of CAG repeat length and age.

188 ed proximity to clinical diagnosis (based on CAG repeat length and current age) and striatal volumes.

189 uced gene proximity, androgen receptor exon1 CAG repeat length and expression of the PIWIL1 gene.

190 /2 Huntington's Disease models, differing in CAG repeat length and genetic background (115 and 250 CA

191 this read-through product is proportional to CAG repeat length and is present in all knock-in mouse m

192 rization (SLiC), to identify linkage between CAG repeat length and nucleotide identity of heterozygou

193 nsistent with the hypothesis that somatic HD CAG repeat length expansions in target tissues contribut

194 Age and CAG repeat length explained variance in longitudinal cha

195 of disease risk and age-of-onset on expanded CAG repeat length in diseases like Huntington's disease

196 lished by December 29, 2013, reporting ATXN2 CAG repeat length in patients with ALS and controls.

197 in insight into how mutant huntingtin (mHtt) CAG repeat length modifies Huntington's disease (HD) pat

198 serum testosterone levels and inversely with CAG repeat length, age and duration of weakness.

199 osis of Huntington's disease, accounting for CAG repeat length, age, and the interaction of CAG repea

200 n of 11 HD participants had known huntingtin CAG repeat length, allowing determination of a burden of

201 In a model adjusted for age, CAG repeat length, and caloric intake, MeDi was not asso

202 had prognostic value, independent of age and CAG repeat length, for predicting subsequent clinical di

203 individually matched with incident cases on CAG repeat length, sex, and age, who were not diagnosed

204 ormulas have been developed based on age and CAG repeat length, to predict when HD motor onset will o

205 Here we show that CAG repeat length-dependent aberrant splicing of exon 1

206 nd protein were not associated with expanded CAG repeat length.

207 discordant for AO after correction for SCA2 CAG repeat length.

208 ssociation between cytosine-adenine-guanine (CAG) repeat length and age at onset of Huntington's dise

209 t, HD age at death is determined by expanded CAG-repeat length and has no contribution from the norma

210 HTT haplotypes were associated with altered CAG-repeat length distribution or residual age at the on

211 e selected behavioral signatures for age and CAG-repeat length that most robustly distinguished betwe

212 elic series of R6/2 mice carrying a range of CAG repeat lengths between 109 and 464.) This analysis r

213 Our data show that extreme CAG repeat lengths in R6/2 mice is paradoxically associa

214 CAG repeat lengths of 36 or greater were observed in six

215 The ATXN2 CAG repeat lengths ranged from 13 to 39 in patients with

216 from an iterative strategy yielded predicted CAG repeat lengths that were significantly positively co

217 HD mouse model, R6/2, carrying two different CAG repeat lengths, and a relatively high degree of over

218 gradient of decreasing pathology with longer CAG repeat lengths, reflecting our previous findings wit

219 m DM1 fibroblasts, all showing different CTG.CAG repeat lengths, thus demonstrating somatic instabili

220 icted by the length of their constitutive HD CAG repeat lengths.

221 amples from HD knock-in mice with increasing CAG repeat lengths.

222 3,086 behavioral traits with seven different CAG-repeat lengths in the huntingtin gene (Htt).

223 of endogenous mouse HTT genes, with variable CAG-repeat lengths.

224 tisense oligonucleotide complementary to the CAG repeat (LNA-CTG) preferentially binds to mutant HTT

225 tificial ZFP chains, designed to bind longer CAG repeats more strongly than shorter repeats.

226 n-enriched RNA from flies expressing a toxic CAG-repeat mRNA (CAG100) and a non-toxic interrupted CAA

227 inates both stress-induced rereplication and CAG repeat mutagenesis.

228 irs motor function in men and is linked to a CAG repeat mutation in the androgen receptor (AR) gene.

229 th human huntingtin protein with an expanded CAG repeat mutation in the juvenile range.

230 The knock-in mice carrying a 72-80 CAG repeat mutation is an accurate genetic model of earl

231 s disease (HD), the size of the expanded HTT CAG repeat mutation is the primary driver of the process

232 determined primarily by the length of the HD CAG repeat mutation, but is also influenced by other mod

233 Since disease-causing CAG repeats occur in transcribed regions, our results su

234 s provide evidence that breakage at expanded CAG repeats occurs due to R-loop formation and reveal tw

235 In addition, expression of an untranslated CAG repeat of pathogenic length conferred neuronal degen

236 rmal expansion in the polyglutamine encoding CAG repeat of the androgen receptor gene.

237 with strong selectivity for CUG repeats over CAG repeats or CAG-CUG duplex RNA.

238 ense oligonucleotides (ASOs) targeted to the CAG repeat region of HTT transcripts have been of partic

239 disorder caused by a toxic expansion in the CAG repeat region of the huntingtin gene.

240 he nontemplate DNA strand at transcribed CTG.CAG repeats remains partially single-stranded in human g

241 l repeat range, supporting the view that the CAG repeat represents a functional polymorphism with dom

242 Convergent transcription through a CAG repeat represents a novel mechanism for triggering a

243 rovide evidence for a pathogenic role of the CAG repeat RNA in polyQ toxicity using Drosophila.

244 Ataxin-3 polyQ protein could also modify the CAG-repeat RNA toxicity.

245 egulation of heat shock protein 70 mitigates CAG-repeat RNA toxicity.

246 cent findings, however, demonstrate that the CAG-repeat RNA, which encodes the toxic polyQ protein, a

247 modifiers of both polyQ protein toxicity and CAG-repeat RNA-based toxicity.

248 e tested the role of the RNA by altering the CAG repeat sequence to an interrupted CAACAG repeat with

249 se damage (e.g., 8-oxo-dG) occurs at or near CAG repeat sequences.

250 r nucleases (ZFNs) that recognize and cleave CAG repeat sequences.

251 exon 1 of human Hdh, and R6/2 mice with 150 CAG repeats show neurological abnormalities by 10 weeks

252 ferences were independent of constitutive HD CAG repeat size and did not correlate with Hdh mRNA leve

253 Expanded CAG repeat size is the primary determinant of age at ons

254 n (all p < 0.001), independent of mutant HTT CAG repeat size.

255 The CAG repeat specifies glutamine, and the expanded polyQ d

256 stone H4 acetylation is required to maintain CAG repeat stability and promote gap-induced sister chro

257 t (<4 months) HD mice harbouring an expanded CAG repeat stretch and age-matched wild type (WT) mice r

258 The genetic cause is an expansion of the CAG repeat stretch in the HTT gene encoding huntingtin p

259 ed characteristics of the gene products with CAG repeats, such as in vitro and in vivo aggregation an

260 an cDNA fragment of the TBP gene with 64 CAA/CAG repeats (TBPQ64).

261 In humans, long CAG repeats tend to expand during transmissions from par

262 The expanded CAG repeat that causes striatal cell vulnerability in Hu

263 SCA7 and SCA17 are caused by expansion of a CAG repeat that encodes a polyglutamine tract in the aff

264 on disease (HD) is caused by an expanded HTT CAG repeat that leads in a length-dependent, completely

265 by the increased propensity of the expanded CAG repeats to form a stem-loop structure.

266 xia type 1 (SCA1), which carries an expanded CAG repeat tract at the endogenous mouse Sca1 locus.

267 5'S)-5',8-cyclo-2'-deoxyadenosine (cdA) in a CAG repeat tract caused CTG repeat deletion exclusively

268 unusual properties alter the organization of CAG repeat tract chromatin.

269 ington's disease (HD), caused by an expanded CAG repeat tract in HTT, genetic variation has been unco

270 dative stresses induce mutagenesis of a long CAG repeat tract in human cells.

271 ed three PMOs to selectively target expanded CAG repeat tracts (CTG22, CTG25 and CTG28), and two PMOs

272 Longer CAG repeat tracts are associated with earlier ages at on

273 Expanded CAG repeat tracts are the cause of at least a dozen neur

274 Unusual physical properties of CAG repeat tracts are thought to contribute to their ins

275 Long CAG repeat tracts cause human hereditary neurodegenerati

276 previously shown that transcription through CAG repeat tracts destabilizes them in a way that depend

277 In human patients and mouse models, CAG repeat tracts display an ongoing instability in neur

278 epitope-tagged Hmo1 selectively precipitates CAG repeat tracts DNAs that range from 26 to 126 repeat

279 we performed a yeast one-hybrid screen using CAG repeat tracts embedded in front of two reporter gene

280 ng a selection assay based on contraction of CAG repeat tracts in human cells, we screened the Prestw

281 Expansions of CAG repeat tracts in the germ line underlie several neur

282 These results show that Hmo1 binds to CAG repeat tracts in vivo and establish the basis of the

283 on enzyme cleavage in or near CGG*CCG or CTG*CAG repeat tracts on their genetic instabilities, both w

284 We report that CAG repeat tracts, embedded in yeast chromosomes, have a

285 .DNA hybrids enhances the instability of CTG.CAG repeat tracts.

286 ich incise DNA adjacent to damage, stabilize CAG repeat tracts.

287 n can promote repeat expansion, using (CTG)*(CAG) repeat tracts in the size range that is typical for

288 transgene locus driving the expression of a CAG repeat transcript (HDL2-CAG) from the strand antisen

289 ygous DCTN1 p.T54I, FUS p.P431L, and HTT (42 CAG repeats) were identified as pathogenic mutations.

290 gh HD pathogenesis is driven by the expanded CAG repeat, whether the mutation influences the expressi

291 mhtt in BACHD mice is encoded by a mixed CAA-CAG repeat, which is stable in both the germline and som

292 ypical +1 shift site, UUC C at the 5' end of CAG repeats, which has some resemblance to the influenza

293 are consistent with segmental motions of the CAG repeat, while also suggesting that the 2AP probe is

294 corrected by the replacement of the expanded CAG repeat with a normal repeat using homologous recombi

295 glutamine disorders caused by expansion of a CAG repeat within the coding regions of the Ataxin-1 and

296 s disease (HD), which are caused by expanded CAG repeats within an allele of the ataxin-3 (ATXN3) and

297 tion by blocking the detrimental activity of CAG repeats within HTT mRNA.

298 HD and MJD are caused by an expansion of CAG repeats within one mRNA allele encoding huntingtin (

299 ession by ss-siRNAs that target the expanded CAG repeats within the mutant allele.

300 a yeast artificial chromosome containing 128 CAG repeats (YAC128) with low-dose memantine blocks extr