ライフサイエンスコーパス: spinocerebellar ataxia type 1

1 elated progressive neurodegeneration seen in spinocerebellar ataxia type 1.

2 l to decipher the pathogenesis mechanisms in spinocerebellar ataxia type 1.

3 ct in Ataxin-1 causes the autosomal dominant spinocerebellar ataxia type 1.

4 omers are the primary drivers of toxicity in Spinocerebellar ataxia type 1.

5 d are supportive of clinical application for spinocerebellar ataxia type 1.

6 cted individuals with identified expansions (spinocerebellar ataxia types 1, 2, 3, 6 and 7), recruite

7 ciation between age at onset and CAG size in spinocerebellar ataxia types 1, 3 and 6.

8 normal alleles in trans in individuals with spinocerebellar ataxia types 1, 6 and 7.

9 Ataxin-1 is a human protein responsible for spinocerebellar ataxia type 1, a hereditary disease asso

10 Except for individuals with spinocerebellar ataxia type 1, age at onset was also inf

11 In particular, spinocerebellar ataxia type 1 and 7 (SCA1 and SCA7) pati

12 y as well, both as a mediator of toxicity in spinocerebellar ataxia type 1 and as a tumor suppressor

13 (CIC) has been implicated in pathogenesis of spinocerebellar ataxia type 1 and cancer in mammals; how

14 et repeat" diseases through mouse models for spinocerebellar ataxia types 1 and 3 and Huntington dise

15 xpanded ATAXIN-1, the protein that underlies spinocerebellar ataxia type 1, forms toxic oligomers and

16 Spinocerebellar ataxia type 1 is a late-onset neurodegen

17 Spinocerebellar ataxia type 1 is an autosomal dominant c

18 Spinocerebellar ataxia type 1 is an autosomal dominant f

19 Spinocerebellar ataxia type 1 is caused by expansion of

20 Spinocerebellar ataxia type 1 is one of nine polyglutami

21 domain of ataxin-1, the protein involved in spinocerebellar ataxia type-1, is the region responsible

22 ptions within the (CAG)n or (CGG)n tracts of spinocerebellar ataxia, type 1 or fragile X syndrome, re

23 yglutamine expansion diseases, which include spinocerebellar ataxia type 1 (SCA1) and Huntington dise

24 Spinocerebellar Ataxia type 1 (SCA1) and Huntington's di

25 e expansion of an unstable CAG repeat causes spinocerebellar ataxia type 1 (SCA1) and several other n

26 To elucidate the pathophysiology of spinocerebellar ataxia type 1 (SCA1) and to evaluate rep

27 degeneration in transgenic mice carrying the spinocerebellar ataxia type 1 (SCA1) gene is modulated b

28 Transgenic mice carrying the spinocerebellar ataxia type 1 (SCA1) gene, a polyglutami

29 whose glutamine-repeat expanded form causes spinocerebellar ataxia type 1 (SCA1) in humans and exert

30 tures of the human neurodegenerative disease spinocerebellar ataxia type 1 (SCA1) in the mouse, we ta

31 Spinocerebellar ataxia type 1 (SCA1) is a dominantly inh

32 Spinocerebellar ataxia type 1 (SCA1) is a dominantly inh

33 Spinocerebellar ataxia type 1 (SCA1) is a dominantly inh

34 Spinocerebellar ataxia type 1 (SCA1) is a fatal neurodeg

35 Spinocerebellar ataxia type 1 (SCA1) is a lethal neurode

36 Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerat

37 Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerat

38 Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerat

39 Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerat

40 Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerat

41 Spinocerebellar ataxia type 1 (SCA1) is a paradigmatic n

42 The neurodegenerative disease Spinocerebellar ataxia type 1 (SCA1) is a polyglutamine

43 Spinocerebellar ataxia type 1 (SCA1) is a relatively rar

44 Spinocerebellar ataxia type 1 (SCA1) is an adult-onset,

45 Spinocerebellar ataxia type 1 (SCA1) is an autosomal dom

46 Spinocerebellar ataxia type 1 (SCA1) is an autosomal dom

47 Spinocerebellar ataxia type 1 (SCA1) is an autosomal dom

48 Spinocerebellar ataxia type 1 (SCA1) is an autosomal dom

49 Spinocerebellar ataxia type 1 (SCA1) is an autosomal dom

50 Spinocerebellar ataxia type 1 (SCA1) is an autosomal dom

51 Spinocerebellar ataxia type 1 (SCA1) is an autosomal dom

52 Spinocerebellar ataxia type 1 (SCA1) is an autosomal dom

53 Spinocerebellar ataxia type 1 (SCA1) is an incurable neu

54 Spinocerebellar ataxia type 1 (SCA1) is one of nine domi

55 Spinocerebellar ataxia type 1 (SCA1) is one of nine inhe

56 Spinocerebellar ataxia type 1 (SCA1) is one of nine inhe

57 Spinocerebellar ataxia type 1 (SCA1) is one of several n

58 Spinocerebellar ataxia type 1 (SCA1) is one of several n

59 Spinocerebellar ataxia type 1 (SCA1) is one of several n

60 Spinocerebellar ataxia type 1 (SCA1) is one such disease

61 Spinocerebellar ataxia type 1 (SCA1) is one such disease

62 ional expansion of CAG repeats at the murine spinocerebellar ataxia type 1 (Sca1) locus.

63 Expressing pathogenic spinocerebellar ataxia type 1 (SCA1) or type 3 (SCA3) pr

64 at risk, making this collection the largest spinocerebellar ataxia type 1 (SCA1) pedigree known.

65 nt studies with a conditional mouse model of spinocerebellar ataxia type 1 (SCA1) suggest that neuron

66 key molecule modulating disease toxicity in spinocerebellar ataxia type 1 (SCA1), a disease caused b

67 ell-based and Drosophila genetic screens, to spinocerebellar ataxia type 1 (SCA1), a disease caused b

68 protein of unknown function associated with spinocerebellar ataxia type 1 (SCA1), a neurodegenerativ

69 , the expanded polyglutamine protein causing spinocerebellar ataxia type 1 (SCA1), aggregates in ubiq

70 r atrophy (SBMA), Huntington's disease (HD), spinocerebellar ataxia type 1 (SCA1), dentatorubral pall

71 entatorubral-pallidoluysian atrophy (DRPLA), spinocerebellar ataxia type 1 (SCA1), Machado-Joseph dis

72 However, in a mouse model of spinocerebellar ataxia type 1 (SCA1), we identify a prev

73 a mouse model of the polyglutamine disorder spinocerebellar ataxia type 1 (SCA1), we tested the hypo

74 eliminates NER, into the TNR mouse model for spinocerebellar ataxia type 1 (SCA1), which carries an e

75 he protein responsible for neurodegenerative spinocerebellar ataxia type 1 (SCA1).

76 eat instability in a knock-in mouse model of spinocerebellar ataxia type 1 (SCA1).

77 or of toxicity in the polyglutamine disease, spinocerebellar ataxia type 1 (SCA1).

78 a polyglutamine tract within ataxin-1 causes spinocerebellar ataxia type 1 (SCA1).

79 ATXN1 mRNA (rAAV.miS1), to a mouse model of spinocerebellar ataxia type 1 (SCA1; B05 mice).

80 rotein aggregates seen in Parkinson disease, spinocerebellar ataxia type 1 (SCA1; ref.

81 polyglutamine protein whose expansion causes spinocerebellar ataxia type-1 (SCA1) and triggers the fo

82 ve disorder protein whose mutant form causes spinocerebellar ataxia type-1 (SCA1).

83 n the early stages of a mouse model of human spinocerebellar ataxia type 1, SCA1, where mice exhibit

84 ion of CAG repeats in ATAXIN1 (ATXN1) causes Spinocerebellar ataxia type 1, the functions of ATXN1 an

85 Two recently published papers on spinocerebellar ataxia type 1, together with studies on

86 as been indicated to be the disease gene for spinocerebellar ataxia type 1, which is also a neurodege