ライフサイエンスコーパス: polyglutamine disease

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

2 reduce lethality in two Drosophila models of polyglutamine disease.

3 nd indicate a novel therapeutic strategy for polyglutamine disease.

4 Drosophila models of Alzheimer's disease and polyglutamine disease.

5 assay that recapitulates several features of polyglutamine disease.

6 d neurodegeneration in a Drosophila model of polyglutamine disease.

7 erones as a potential therapeutic target for polyglutamine diseases.

8 an important role in the pathophysiology of polyglutamine diseases.

9 is thus regarded as the most unstable of the polyglutamine diseases.

10 ons (NIIs), a hallmark of SBMA and the other polyglutamine diseases.

11 ng therapeutic strategies for this and other polyglutamine diseases.

12 clearance might underlie the pathogenesis of polyglutamine diseases.

13 ease, reminiscent of juvenile forms of other polyglutamine diseases.

14 and Parkinson's disease, prion disorders and polyglutamine diseases.

15 o altering the progression of this and other polyglutamine diseases.

16 RAN translation may also contribute to other polyglutamine diseases.

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

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

19 structure/function and neurodegeneration in polyglutamine diseases.

20 inal and bulbar muscular atrophy and related polyglutamine diseases.

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

22 sregulation is an important early feature of polyglutamine diseases.

23 ations for the pathogenesis and treatment of polyglutamine diseases.

24 mportant for unravelling the pathogenesis of polyglutamine diseases.

25 ction, a mechanism that may apply broadly to polyglutamine diseases.

26 onal dysfunction and/or neurodegeneration in polyglutamine diseases.

27 ecular pathogenesis of SCA1 as well as other polyglutamine diseases.

28 for histone deacetylase (HDAC) inhibitors in polyglutamine diseases.

29 e pathways may be effective for treatment of polyglutamine diseases.

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

31 diseases may be much broader than HD or even polyglutamine diseases.

32 mentia, Huntington's disease (HD), and other polyglutamine diseases.

33 l intervention in SBMA and potentially other polyglutamine diseases.

34 rse neurodegenerative diseases including the polyglutamine diseases.

35 plicing may contribute to the progression of polyglutamine diseases.

36 es a new treatment strategy for HD and other polyglutamine diseases.

37 Hsp70 is a potent suppressor of both polyglutamine disease and Parkinson's disease in Drosoph

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

39 Ggamma is not a viable therapeutic target in polyglutamine disease and that overall proteasome functi

40 s affected in Huntington's disease and other polyglutamine diseases and by discerning whether gene ex

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

42 omparing modifiers isolated in the models of polyglutamine diseases and in a Drosophila model of tauo

43 xpanded protein is a unifying feature of CAG/polyglutamine diseases and may be initiated or catalyzed

44 gest a therapeutic approach for treatment of polyglutamine diseases and provide the potential for yea

45 similar role for glial dysfunction in other polyglutamine diseases and SCAs.

46 findings may be relevant to the treatment of polyglutamine diseases and, perhaps, to other neurodegen

47 Polyglutamine diseases are a class of dominantly inherit

48 Polyglutamine diseases are a family of neurodegenerative

49 Polyglutamine diseases are a family of nine neurodegener

50 Polyglutamine diseases are a major cause of neurodegener

51 Polyglutamine diseases are caused by an expanded glutami

52 Polyglutamine diseases are caused by CAG expansions in d

53 The current working hypothesis is that polyglutamine diseases are caused by misfolding and aggr

54 Polyglutamine diseases are dominantly inherited neurodeg

55 Polyglutamine diseases are inherited neurodegenerative d

56 ease, amyotrophic lateral sclerosis, and the polyglutamine diseases, are characterized by intracellul

57 roteins may also be present in the tissue of polyglutamine diseases as a result of frameshifting of t

58 loss of neurite outgrowth and cell death in polyglutamine diseases, as these phenotypes were partial

59 ew leads for therapeutic development for the polyglutamine diseases based on their efficacy in mammal

60 into the cell specificity of pathology for a polyglutamine disease by relating SCA7-induced retinal d

61 Ggamma could contribute to UPS impairment in polyglutamine diseases by suppressing the proteasomal ca

62 Polyglutamine diseases comprise a class of familial neur

63 damental unanswered question in the field of polyglutamine diseases concerns the pathophysiology of n

64 The polyglutamine diseases consist of nine neurodegenerative

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

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

67 For polyglutamine diseases generally, the findings support a

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

69 n the striatum of various knock-in models of polyglutamine diseases highlights the role of trans-acti

70 Experimental models of polyglutamine disease implicate the nucleus in pathogene

71 This disease is unusual among the polyglutamine diseases in that it involves lower motor a

72 Polyglutamine diseases include at least nine neurodegene

73 The polyglutamine diseases include at least nine neurodegene

74 HSP27 is also found in cell models of other polyglutamine diseases, including Huntington disease as

75 The polyglutamine diseases, including Huntington's disease (

76 nisms of neurodegeneration in the CAG repeat polyglutamine diseases, including Spinal and Bulbar Musc

77 Polyglutamine diseases, including spinocerebellar ataxia

78 The family of polyglutamine diseases is characterized by the presence

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

80 A pathological hallmark of polyglutamine diseases is the presence of inclusions or

81 A hallmark of these so-called polyglutamine diseases is the presence of ubiquitylated

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

83 pendent manner, suggesting that pathology in polyglutamine disease may result from cellular depletion

84 In polyglutamine disease models, CHIP has been considered a

85 to the selective neurodegeneration in other polyglutamine diseases or not.

86 These studies encompass the polyglutamine diseases, Parkinson's disease, Alzheimer's

87 ion for further studies as a therapeutic for polyglutamine diseases, particularly as it is an establi

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

89 P-43 function may play an unexpected role in polyglutamine disease pathogenesis.

90 normal aging processes might be involved in polyglutamine disease pathogenesis.

91 In all polyglutamine diseases, polyglutamine-expanded proteins

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

93 regulates the activity of a DUB, ataxin-3, a polyglutamine disease protein implicated in protein qual

94 t host protein context is the key arbiter of polyglutamine disease protein toxicity.

95 Finally, studies of a third polyglutamine disease protein, ataxin-1, reveal unexpect

96 Here we show that the polyglutamine disease protein, ataxin-3, binds and cleav

97 ations, and reporter assays to show that the polyglutamine disease protein, ataxin-3, interacts with

98 cell-based approaches we establish that the polyglutamine disease protein, ataxin-3, is a poly-ubiqu

99 eurodegeneration induced by human pathogenic polyglutamine disease protein.

100 ve effects produced by expression of a human polyglutamine disease protein.

101 Among the polyglutamine diseases, protein folding and histone acet

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

103 he mechanisms of pathology for the family of polyglutamine disease proteins are unknown; however, rec

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

105 e of these inclusions in the pathogenesis of polyglutamine diseases remains unclear.

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

107 sed, the neurodegeneration in SCA1 and other polyglutamine diseases selectively involves a few neuron

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

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

110 We have developed a transgenic model of the polyglutamine disease spinal and bulbar muscular atrophy

111 loss in the inherited ataxias, including the polyglutamine disease spinocerebellar ataxia type 3 (SCA

112 se pathogenesis to ubiquitin pathways in the polyglutamine disease spinocerebellar ataxia type 3 (SCA

113 In the polyglutamine disease spinocerebellar ataxia type 7 (SCA

114 ) as a candidate mediator of toxicity in the polyglutamine disease, spinocerebellar ataxia type 1 (SC

115 ns formed in a cell culture model of another polyglutamine disease, spinocerebellar ataxia type 3.

116 may be a common mechanism of pathogenesis in polyglutamine diseases such as Huntington disease and sp

117 a primary cause of cellular pathogenesis in polyglutamine diseases such as Huntington disease; the r

118 In polyglutamine diseases such as X-linked spinobulbar musc

119 Conditional mouse models of polyglutamine diseases, such as Huntington's disease (HD

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

121 primary site of protein aggregation in many polyglutamine diseases, suggesting a central role in pat

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

123 In SCA1 and several other polyglutamine diseases, the expanded protein aggregates

124 In all known polyglutamine diseases, the glutamine expansion confers

125 or class of neurodegenerative disorders, the polyglutamine diseases, to show reduced polyglutamine ag

126 fer toxicity to the mRNA and, in the case of polyglutamine diseases, to the encoded protein.

127 Therefore, in HD, as well as in other polyglutamine diseases, tTG is unlikely to play a role i

128 Drosophila model for Huntington's and other polyglutamine diseases was used to screen for genetic fa

129 important development in the study of such "polyglutamine" diseases was the realization that merely

130 Using a Drosophila melanogaster model of polyglutamine disease, we show that directed expression

131 as allowed subclassification into translated polyglutamine diseases, which are due to CAG repeat expa

132 erstanding of the role of protein folding in polyglutamine disease with emerging evidence that altera

133 G repeats are often used to create models of polyglutamine diseases yet are very rare in humans where