ライフサイエンスコーパス: MND

1 MND induced apoptosis, inhibited migration and invasion,

2 MND patients commonly displayed an abnormal pattern duri

3 genual anterior cingulate cortex (ACC) in 13 MND and eight control subjects.

4 clinical similarity between this disease and MND seen in other countries.

5 nd the relationship between this disease and MND seen in the rest of the world are still uncertain.

6 ORF72 expansions: 35 had FTD, 16 had FTD and MND, 30 had MND, and three had no clinical phenotype.

7 (47 patients [15 with FTD, nine with FTD and MND, and 23 with MND] and three carriers who had no clin

8 m (40 patients [20 with FTD, 12 with FTD and MND, and eight with MND]), and blood (47 patients [15 wi

9 om 41 patients [21 with FTD, 11 with FTD and MND, and nine with MND]), cerebellum (40 patients [20 wi

10 re the most common genetic cause of FTLD and MND identified to date.

11 ssion from a pair of vectors, L-tNGFR-SN and MND-tNGFR-SN, indicated that only 1.04% of the CCE cells

12 ens are associated with undernourishment and MNDs.

13 ons were not significantly different between MND and control cases, although there were trends toward

14 c FTLD subgroup, and 286 controls or between MND cases and controls.

15 tion in RNA processing and regulation, cause MNDs and place the dysregulation of RNA pathways at the

16 ked by the discovery of the genes that cause MNDs.

17 that were modified with the human ADA cDNA (MND-ADA) gamma-retroviral vector after conditioning with

18 series suggest that pathologically classical MND on Guam may occur independently of neurofibrillary d

19 ited but mostly sporadic, is the most common MND.

20 or more chronic micronutrient deficiencies (MNDs).

21 ol for studies of motor neuron degeneration (MND).

22 lthough for most cancer patients who develop MND the occurrence of both disorders is probably coincid

23 Group 3: Six patients developed MND resembling amyotrophic lateral sclerosis between 47

24 sease, is a late-onset motor neuron disease (MND) caused by an abnormal expansion of the CAG repeat i

25 Motor neuron disease (MND) is a common neurodegenerative condition for which t

26 ompounds contribute to motor neuron disease (MND) is supported by association of paraoxonase 1 polymo

27 reports indicate that motor neuron disease (MND) may rarely be associated with systemic cancer.

28 progressive and fatal motor neuron disease (MND) similar to amyotrophic lateral sclerosis (ALS).

29 ssion in patients with motor neuron disease (MND), as tools for future clinical trials, and to probe

30 ondrial dysfunction in motor neuron disease (MND), but the molecular basis of these abnormalities is

31 and 259 patients with motor neuron disease (MND), for whom genomic DNA was available, were investiga

32 stological features of motor neuron disease (MND), the term FTLD-MND is used.

33 We revealed that motor-neuron disease (MND)-linked RNA-binding proteins (RBPs), TDP-43, FUS, an

34 ral dementia (FTD) and motor neuron disease (MND).

35 somal dominant form of motor neuron disease (MND).

36 TLD-U) with or without motor neuron disease (MND).

37 nctionally abnormal in motor neuron disease (MND, amyotrophic lateral sclerosis), but the nature of t

38 In 2003 the Motor Neurone Disease (MND) Association, together with The Wellcome Trust, fund

39 the high incidence of motor neurone disease (MND) on Guam, and the relationship between this disease

40 tly, 32 patients with motor neurone disease (MND) were studied to identify factors associated with an

41 iologically confirmed motor neurone disease (MND), in whom communication problems were an early and d

42 In motor neurone disease (MND), respiratory muscle weakness causes substantial mor

43 osis (ALS, also called motor neuron disease, MND) are severe neurodegenerative diseases that show con

44 Motor neuron diseases (MNDs) are neurodegenerative disorders that lead to paral

45 Motor neuron diseases (MNDs) are progressive neurodegenerative disorders charac

46 and cellular bases of motor neuron diseases (MNDs) are still poorly understood.

47 s and dysfunctions in motor neuron diseases (MNDs) such as amyotrophic lateral sclerosis (ALS).

48 sis (ALS) and related motor neuron diseases (MNDs).

49 practice for minor neurocognitive disorder (MND) as well as HAD, despite uncertainty about their acc

50 in mice, which developed normally, displayed MND-like phenotypes after 10 months of age, including ex

51 peroxide dismutase 1 (SOD1) related familial MND (fMND).

52 creation of a national DNA Bank specific for MND.

53 d 54.7%, and sensitivity and specificity for MND were 64.3% and 66.0%.

54 d 77.9%, and sensitivity and specificity for MND were estimated at 42.0% and 91.2%.

55 d that long terminal repeat transcripts from MND-CAT-SN are >80 times more abundant than the L-CAT-SN

56 mporal dementia (FTD) with a sister with FTD+MND and the other in a case of progressive non-fluent ap

57 ize-72) in a cohort of individuals with FTD, MND, both these diseases, or no clinical phenotype.

58 D overlapping with motor neuron disease [FTD-MND]), followed by a meta-analysis of the entire dataset

59 wed typical histological appearances of FTLD-MND in two cases and of FTLD-U in one case.

60 FTLD-U or FTLD-MND should be considered in the differential diagnosis o

61 of motor neuron disease (MND), the term FTLD-MND is used.

62 h no family history, who showed a mixed FTLD/MND picture and A324T change in exon 9 was found in two

63 We present a series of 45 cases of Guamanian MND, which reaffirm the clinical similarity between this

64 ions: 35 had FTD, 16 had FTD and MND, 30 had MND, and three had no clinical phenotype.

65 However, MND failed to directly inhibit EGFR or other related rec

66 l layers V (P = 0.003) and VI (P = 0.001) in MND cases compared with controls.

67 neuroimaging and cognitive abnormalities in MND.

68 layer V in the PMC, the DLPFC and the ACC in MND subjects compared with controls [t (19) = 2.91, P =

69 dwide and significantly increase activity in MND genetic research.

70 ss in the PMC and in other cortical areas in MND.

71 tive axonal transport has been implicated in MND and other forms of poly-Q disease.

72 ducted of the clinical application of NIV in MND among consultant neurologists in the UK.

73 ment of denervation over short timescales in MND and enables investigation of patterns of disease spr

74 ssed mutated RBP aggregation and toxicity in MND Drosophila models.

75 IVmnd-2-infected MNDs than SIVmnd-1-infected MNDs.

76 able between SIVmnd-1- and SIVmnd-2-infected MNDs and to those observed in uninfected animals, with t

77 cell counts in chronically SIVmnd-2-infected MNDs than SIVmnd-1-infected MNDs.

78 -producing CD8+ T cells in SIVmnd-2-infected MNDs.

79 n CD4+ T cell counts and VLs in SIV-infected MNDs could be established.

80 erved in another natural host, the mandrill (MND), we conducted a cross-sectional survey of the 23 SI

81 We have previously reported the modified MND LTR (myeloproliferative sarcoma virus enhancer, nega

82 ES and EC cells transduced with the modified MND-CAT-SN vector than in those transduced with L-CAT-SN

83 of mitochondrial dysfunction in the cause of MND.

84 tion has confirmed the clinical diagnosis of MND-dementia.

85 EGFR revealed that the biological effects of MND could be abrogated by pertussis toxin.

86 atients with or without clinical evidence of MND.

87 Pathologically, the classical features of MND were seen in Guamanian Chamorro cases including ubiq

88 However, the occurrence of MND among the indigenous Chamorros of Guam is distinguis

89 ateral sclerosis (ALS) and the occurrence of MND in OP compound-induced delayed neuropathy (OPIDN), i

90 The onset of MND was delayed in these mice compared to the original G

91 In group 2, the proximate onset of MND with the diagnosis of cancer or its recurrence, its

92 ransport during the onset and progression of MND in a line of mice that contained approximately 30% f

93 The classical signs of MND, including wasting, fasciculations and severe bulbar

94 patient with clinical features suggestive of MND but additional cardiac and metabolic symptoms.

95 tion and the clinicopathological syndrome of MND may occur in parallel, observations from this series

96 iation with the dementia/aphasia syndrome of MND suggests that the neural substrate underlying verb r

97 sative gene of a clinically diverse group of MNDs including amyotrophic lateral sclerosis (ALS), atyp

98 understanding of the underlying mechanism of MNDs and aid in the development of effective treatments.

99 With the development of mouse models of MNDs, a noninvasive neuroimaging modality capable of det

100 lculated for each scale as a test for HAD or MND.

101 was similar to that from the MND-tNGFR-SN or MND-EGFP-SN vector in nearly all cells, suggesting that

102 ng functional motor neurons in ALS and other MNDs.

103 NTE pathway disturbances contribute to other MNDs including ALS, and supports the role of NTE abnorma

104 -yl)methanone O-2-(diethylamino)ethyl oxime (MND) exhibited the best safety profile.

105 ase SCYL1, causes an early onset progressive MND with characteristic features of amyotrophic lateral

106 ree patients developed a rapidly progressive MND, less prominent symptoms of involvement of other are

107 ctors using either a viral-derived promoter, MND, or the human proximal WAS promoter (WS1.6) for huma

108 and vectors based on our previously reported MND backbone, which has alterations to address three vir

109 These findings support the notion that MND should be considered a multisystem disorder.

110 Mechanistic studies revealed that MND strongly abrogated EGF-induced proliferation, migrat

111 The MND-ADA vector was persistently detected in PBMCs (vecto

112 In group 1, the MND associated with the anti-Hu antibody is unequivocall

113 ed a higher frequency of expression from the MND LTR (20% to 80%) in hematopoietic cells of all linea

114 Expression from the MND LTR reached 88% in thymic T lymphocytes and 54% in s

115 sed slightly to 3% while expression from the MND-EGFP-SN vector persisted in 80% of the cells.

116 rter expression was similar to that from the MND-tNGFR-SN or MND-EGFP-SN vector in nearly all cells,

117 suggest further clinical development of the MND-huWASp LV for a future clinical trial for WAS.

118 tor in nearly all cells, suggesting that the MND vectors are far less susceptible to position-depende

119 Therefore, the MND vector offers an improved vehicle for reliable gene

120 ssion in only 5% of the population while the MND-EGFP-SN vector drove expression in 88% of the cells.

121 pressed the cell surface reporter, while the MND-tNGFR-SN vector drove expression in 99.54% of the CC

122 non-Chamorros who resided on Guam when their MND symptoms occurred.

123 mechanism whereby mutations in SOD1 lead to MND remains enigmatic, we asked whether NF inclusions in

124 Together, MND represents a new nonquinazoline potential drug candi

125 Today the primary focus of the UK MND DNA Bank still remains to identify causative and dis

126 vey of the 23 SIV-infected and 25 uninfected MNDs from the only semifree colony of mandrills availabl

127 fic, nonconserved NTE mutations in unrelated MND patients indicates NTE's importance in maintaining a

128 Therefore, the modified retroviral vector, MND, achieves higher net levels of expression due to a g

129 with FTD, nine with FTD and MND, and 23 with MND] and three carriers who had no clinical phenotype).

130 ith FTD, 12 with FTD and MND, and eight with MND]), and blood (47 patients [15 with FTD, nine with FT

131 = 72); and 4) familial and sporadic FTD with MND (n = 40).

132 of which were donated by people living with MND, family members and non-related controls, accompanie

133 with FTD, 11 with FTD and MND, and nine with MND]), cerebellum (40 patients [20 with FTD, 12 with FTD

134 ior increased over 4 months in patients with MND (right: 10.2%, 95% CI 2.0% to 18.4%, p=0.017; left:

135 probably coincidental, in some patients with MND a careful search for an underlying cancer is warrant

136 Twenty-nine patients with MND and 22 age-matched and gender-matched healthy contro

137 We have encountered 14 patients with MND and cancer who formed three distinct groups.

138 Patients with MND had 30% higher relative T2 muscle signal than contro

139 evious surveys showed that few patients with MND received NIV.

140 ransplantation of stem cells transduced with MND-huWASp LV resulted in sustained, endogenous levels o

141 while 99.89% of the F9 cells transduced with MND-tNGFR-SN showed expression.

142 poradic and familial FTLD-U with and without MND and expand this disease spectrum to include reported

143 f familial and sporadic FTLD with or without MND.