ライフサイエンスコーパス: polyglutamine expansion

1 erived ataxin-7, which contains a pathogenic polyglutamine expansion.

2 bberant protein interactions mediated by the polyglutamine expansion.

3 C terminal to amino acid 221 to include the polyglutamine expansion.

4 enerative disorder caused by ataxin-3 with a polyglutamine expansion.

5 Huntingtin (Htt), that contains an abnormal polyglutamine expansion.

6 uclear factor or process is disrupted by the polyglutamine expansion.

7 dels of tauopathy, amyloid beta-peptide, and polyglutamine expansion.

8 ease (HD) is expression of huntingtin with a polyglutamine expansion.

9 e epitope of human huntingtin is enhanced by polyglutamine expansion.

10 f many neurodegenerative disorders caused by polyglutamine expansion.

11 ited neurodegenerative disorders result from polyglutamine expansions.

12 1 hereditary neurological diseases involving polyglutamine expansions.

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

14 Although polyglutamine expansion accelerates protein aggregation,

15 Our findings suggest that polyglutamine expansion alters androgen receptor functio

16 N-terminus of mutant huntingtin (htt) has a polyglutamine expansion and forms neuronal aggregates in

17 cytoplasmic inclusions, which increased with polyglutamine expansion and with time after transfection

18 ne, we demonstrate that, while determined by polyglutamine expansion, ataxin-1 aggregation is noticea

19 Concomitantly, polyglutamine expansion attenuates the formation and fun

20 itors inhibit oxidative death independent of polyglutamine expansions by activating an Sp1-dependent

21 In at least nine inherited diseases polyglutamine expansions cause neurodegeneration associa

22 The polyglutamine expansion causes huntingtin to interact ab

23 Polyglutamine expansion causes Huntington disease (HD) a

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

25 While the mechanism(s) by which polyglutamine expansion causes neurodegeneration in each

26 To elucidate how polyglutamine expansion causes neuronal dysfunction, we

27 In Huntington disease (HD), polyglutamine expansion causes the disease protein hunti

28 s are decreased in disease suggests that the polyglutamine expansion contributes to disease by both a

29 We found that polyglutamine expansion decreased ATXN7 occupancy, which

30 onsible for Spinocerebellar ataxia type 3, a polyglutamine expansion disease, represents one of such

31 se data support a CBP-sequestration model of polyglutamine expansion disease.

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

33 This disease and other polyglutamine expansion diseases are characterized by la

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

35 tly proposed mechanisms of neuronal death in polyglutamine expansion diseases include activation of c

36 o its role in the molecular pathology of the polyglutamine expansion diseases, mutations of the prote

37 The dominant polyglutamine expansion diseases, which include spinocer

38 nesis and selective neuronal degeneration of polyglutamine expansion diseases.

39 regates that have been identified in the CAG/polyglutamine expansion diseases.

40 elationship between this disease and the CAG/polyglutamine expansion diseases.

41 HD is one of nine polyglutamine expansion diseases.

42 nce AR SUMOylation may be of clinical use in polyglutamine expansion diseases.

43 e therapy for Huntington's disease and other polyglutamine-expansion diseases.

44 e those seen in models of Kennedy disease, a polyglutamine expansion disorder caused by a CAG repeat

45 se Spinocerebellar ataxia type 1 (SCA1) is a polyglutamine expansion disorder characterized by the de

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

47 ntington disease patients (and perhaps other polyglutamine expansion disorders).

48 nd neurodegenerative diseases, including the polyglutamine expansion disorders, because of its abilit

49 is increasingly implicated in the biology of polyglutamine expansion disorders, Parkinson's disease a

50 therapeutic target for treating HD and other polyglutamine expansion disorders.

51 Here, we find that polyglutamine expansions disrupted the global balance of

52 Data from the present study demonstrate that polyglutamine expansion does not dramatically impair pro

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

54 nd genetic studies provide evidence that the polyglutamine expansion enhances interactions that are n

55 ing and immunoprecipitation assays show that polyglutamine expansion enhances the interaction of N-te

56 Huntington's disease (HD) is caused by polyglutamine expansion (exp) in huntingtin (Htt).

57 Huntington's disease (HD) is caused by polyglutamine expansion (exp) in huntingtin.

58 c features, we have examined the behavior of polyglutamine expansions expressed in Caenorhabditis ele

59 hology of expanded CAG repeat disorders with polyglutamine expansions expressed within the affected p

60 Whereas polyglutamine expansion-expressing animals with WT therm

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

62 CAG/polyglutamine expansion has been shown to form the molec

63 intracellular consequences of expression of polyglutamine expansion have been the object of intensiv

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

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

66 Our results demonstrate that polyglutamine expansion impairs the ability of huntingti

67 Polyglutamine expansion in androgen receptor (AR) is res

68 inant neurodegenerative disorder caused by a polyglutamine expansion in ataxin-3 (ATX3; MJD1) protein

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

70 SCA3, a neurodegenerative disorder caused by polyglutamine expansion in ataxin-3.

71 Polyglutamine expansion in ATXN1 favours the formation o

72 Polyglutamine expansion in certain proteins causes neuro

73 (HD), which is a movement disorder caused by polyglutamine expansion in Htt.

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

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

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

77 However, how polyglutamine expansion in huntingtin promotes glutamate

78 corepressor C-terminal binding protein, and polyglutamine expansion in huntingtin reduced this inter

79 Huntington disease is caused by polyglutamine expansion in huntingtin, a 350 kD protein

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

81 used by a genetic mutation that results in a polyglutamine expansion in huntingtin.

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

83 minant neurodegenerative disease caused by a polyglutamine expansion in huntingtin.

84 The mechanism by which polyglutamine expansion in Huntington's disease (HD) res

85 Huntingtin, a protein altered by polyglutamine expansion in Huntington's disease (Httexp)

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

87 herited neurodegenerative diseases caused by polyglutamine expansion in the affected proteins.

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

89 Polyglutamine expansion in the androgen receptor (AR) ca

90 t in the huntingtin (Htt) protein; a similar polyglutamine expansion in the androgen receptor (AR) ca

91 bulbar muscular atrophy) is caused by a CAG/polyglutamine expansion in the androgen receptor (AR) ge

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

93 y progressive motor neuron disease caused by polyglutamine expansion in the androgen receptor (AR).

94 is a neurodegenerative disorder caused by a polyglutamine expansion in the androgen receptor (AR).

95 rophy and Huntington's disease are caused by polyglutamine expansion in the androgen receptor and hun

96 Polyglutamine expansion in the androgen receptor causes

97 Polyglutamine expansion in the androgen receptor, causin

98 bulbar muscular atrophy (SBMA) is caused by polyglutamine expansion in the androgen receptor, which

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

100 Huntington's disease (HD) is caused by a polyglutamine expansion in the disease protein huntingti

101 Polyglutamine expansion in the exon 1 domain of huntingt

102 gene huntingtin (HTT) that is reflected by a polyglutamine expansion in the Htt protein.

103 We conclude that a small polyglutamine expansion in the human alpha 1A calcium ch

104 The polyglutamine expansion in the huntingtin (htt) protein

105 lmark of HD is neurodegeneration caused by a polyglutamine expansion in the huntingtin (htt) protein

106 a neurodegenerative disorder associated with polyglutamine expansion in the huntingtin (htt) protein.

107 Huntington's disease (HD) is caused by a polyglutamine expansion in the Huntingtin (Htt) protein.

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

109 nant neurodegenerative disorder, caused by a polyglutamine expansion in the huntingtin protein (htt).

110 Polyglutamine expansion in the huntingtin protein is the

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

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

113 Polyglutamine expansion in the N terminus of huntingtin

114 Huntington disease (HD) is caused by polyglutamine expansion in the N terminus of huntingtin

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

116 The Huntington's disease (HD) mutation is a polyglutamine expansion in the N-terminal region of hunt

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

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

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

120 erited neurodegenerative disease caused by a polyglutamine expansion in the protein huntingtin (Htt).

121 odegenerative disorder caused by an abnormal polyglutamine expansion in the protein Huntingtin (Htt).

122 inherited neurological disorder caused by a polyglutamine expansion in the protein huntingtin and is

123 se, a neurodegenerative disorder caused by a polyglutamine expansion in the protein huntingtin.

124 (SCA17), a neurological disease caused by a polyglutamine expansion in the TATA-binding protein (TBP

125 inherited neurodegenerative diseases share a polyglutamine expansion in their respective disease prot

126 Polyglutamine expansions in huntingtin, which causes Hun

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

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

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

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

131 HD is caused by polyglutamine expansions in the huntingtin (htt) protein

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

133 SCA2 results from a poly(Q) (polyglutamine) expansion in the cytosolic protein ataxin

134 neurodegenerative disease linked to a polyQ (polyglutamine) expansion in the huntingtin protein.

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

136 urodegenerative diseases are caused by a CAG/polyglutamine expansion, including spinal and bulbar mus

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

138 gain further insights into the mechanisms of polyglutamine expansion-induced cell death, the Affymetr

139 Knowledge regarding the pathophysiology of polyglutamine-expansion-induced protein dysfunction is a

140 ediates its binding to huntingtin, and (iii) polyglutamine expansion interferes with the ability of h

141 huntingtin interacts with MLK2, whereas the polyglutamine expansion interferes with this interaction

142 Polyglutamine expansion is a hallmark of nine neurodegen

143 erturbation in a target protein induced by a polyglutamine expansion is also discussed.

144 Polyglutamine expansion is central for determining solub

145 Polyglutamine expansion is the cause of several neurodeg

146 nerative diseases, including those caused by polyglutamine expansion, is the formation of ubiquitin (

147 t is dependent on mutant huntingtin dose and polyglutamine expansion; many neurons die without formin

148 y (SBMA) is a motor neuron disease caused by polyglutamine expansion mutation in the androgen recepto

149 Huntington's disease (HD) is caused by a polyglutamine expansion mutation in the huntingtin prote

150 ssive neurodegenerative disorder caused by a polyglutamine expansion near the N-terminus of huntingti

151 ynamics and is a candidate disease sensor in polyglutamine expansion neurodegeneration.

152 , has been implicated in the pathogenesis of polyglutamine expansion neurodegenerative disease.

153 ction of ataxin-2 and the mechanism by which polyglutamine expansion of ataxin-2 causes neurodegenera

154 ction of ataxin-2 and the mechanism by which polyglutamine expansion of ataxin-2 causes neurodegenera

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

156 protein constructs to examine the effects of polyglutamine expansion on protein aggregation, proteaso

157 growth properties of mutant huntingtin with polyglutamine expansions or mutant SOD1 (G85R/G93A) to e

158 Our results suggest that polyglutamine expansions perturb transcription of CREB/C

159 n, is affected by the mutant huntingtin with polyglutamine expansion (polyQ-htt).

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

161 heat-shock proteins also delay the onset of polyglutamine-expansion protein aggregation, suggesting

162 Here we show, in animals expressing polyglutamine expansion proteins and mutant SOD-1(G93A)

163 the accumulation of SCA8 polyalanine and DM1 polyglutamine expansion proteins in previously establish

164 erminal mutant Huntingtin fragment or simple polyglutamine expansion proteins.

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

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

167 How polyglutamine expansions render the resulting proteins t

168 In addition to polyglutamine expansion, requirements for development of

169 ted with decreased clearance of protein with polyglutamine expansion, the accumulation of p62 in neur

170 l-length HD gene expression and differential polyglutamine expansion with possible pathophysiological

171 Polyglutamine expansion within the androgen receptor (AR

172 Polyglutamine expansion within the exon1 of huntingtin l

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

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

175 is a neurodegenerative disorder caused by a polyglutamine expansion within the huntingtin protein.

176 untington's disease is caused by an abnormal polyglutamine expansion within the protein huntingtin an

177 Our results raise the possibility that polyglutamine expansions within ataxin-2 cause neurodege

178 Polyglutamine expansions within different proteins are a