ライフサイエンスコーパス: prion disease

1 idered when treating patients with suspected prion disease.

2 e response accelerated the onset of clinical prion disease.

3 al activation and function may have merit in prion disease.

4 n many neurodegenerative diseases, including prion disease.

5 itochondrial processes may be altered during prion disease.

6 etically validated therapeutic hypothesis in prion disease.

7 me, specifically discriminates patients with prion disease.

8 l role for complement-regulatory proteins in prion disease.

9 P(Sc) with reduced sialylation did not cause prion disease.

10 oped clinical neurologic signs suggestive of prion disease.

11 in 29 healthy controls and 67 patients with prion disease.

12 utic implications through the examination of prion disease.

13 misfolded protein seeds in a murine model of prion disease.

14 disease (PD), Huntington's disease (HD), and prion disease.

15 current non-tissue based diagnostic tests of prion disease.

16 ent to the minimum amount needed to initiate prion disease.

17 en several key events in the pathogenesis of prion disease.

18 There is no well-established trial method in prion disease.

19 Q/K222 goats showed any evidence of clinical prion disease.

20 hlight the role of proteolytic processing in prion disease.

21 ty of the loop also confer susceptibility to prion disease.

22 rimental evidence that dogs are resistant to prion disease.

23 ransition from presymptomatic to symptomatic prion disease.

24 ies of PrP(Sc), neuropathology, and clinical prion disease.

25 mptoms and may even be the primary driver of prion disease.

26 degeneration and hinder plaque formation in prion disease.

27 f neuropathological findings associated with prion disease.

28 the potential impact of sequence variants on prion disease.

29 PrP oligomers may be a cause of toxicity in prion disease.

30 general, as a promising path forward against prion disease.

31 able pancreatic toxicity in a mouse model of prion disease.

32 may offer independent paths forward against prion disease.

33 ectives on the role of familial mutations in prion disease.

34 ved from the brains of mice dying from M1000 prion disease.

35 AF1 might constitute a therapeutic target in prion disease.

36 be a biomarker for preclinical diagnosis of prion disease.

37 can be performed to aid in the diagnosis of prion disease.

38 s, miR-148a-3p, miR-186-5p, miR-30e-3p, with prion disease.

39 egy for the treatment or prevention of human prion disease.

40 -Jakob disease (sCJD) is the prevalent human prion disease.

41 in, leading us to question the role of fH in prion disease.

42 ent of ultrasensitive methods for diagnosing prion disease.

43 an diseases, such as Alzheimer's disease and prion diseases.

44 synuclein aggregates, akin to what occurs in prion diseases.

45 c), the causative agent of neurodegenerative prion diseases.

46 ion test in the broad phenotypic spectrum of prion diseases.

47 cytes may not be just innocent bystanders in prion diseases.

48 milar mechanism by which prions propagate in prion diseases.

49 incurable diseases including Alzheimer's and prion diseases.

50 onformers from Alzheimer's, Parkinson and/or Prion diseases.

51 ansmissible proteinopathies rather than true prion diseases.

52 istic waveforms do not occur in all types of prion diseases.

53 rP(Sc) is key to unraveling the pathology of prion diseases.

54 isorders, and is particularly conspicuous in prion diseases.

55 lateral sclerosis with dementia, as well as prion diseases.

56 t constitute a novel therapeutic approach to prion diseases.

57 tial therapeutic target for the treatment of prion diseases.

58 ases, as well as frontotemporal dementia and prion diseases.

59 ein (PrP(Sc)) have been associated with many prion diseases.

60 s for animal and human health against animal prion diseases.

61 cies transmission barriers characteristic of prion diseases.

62 (PrP) is a critical step in the pathology of prion diseases.

63 h this as a promising therapeutic target for prion diseases.

64 es, including Huntington's, Alzheimer's, and prion diseases.

65 d improve mechanistic understanding of human prion diseases.

66 that plays a key role in the pathogenesis of prion diseases.

67 miRNAs have previously been associated with prion diseases.

68 iated with Alzheimer's, Parkinson's, and the prion diseases.

69 lzheimer's, Parkinson's, type 2 diabetes and prion diseases.

70 ed isoform PrP(Sc) is the causative agent of prion diseases.

71 viduals (19 male and 15 female), and 37 with prion disease (22 male and 15 female).

72 All patients with a final diagnosis of non-prion disease (71 CSF and 67 OM samples) had negative RT

73 successful clinical studies in patients with prion diseases, a 10-y investment to understand its mech

74 akob disease is the most common of the human prion diseases, a group of rare, transmissible, and fata

75 In prion diseases, a major issue in therapeutic research is

76 lopathy of deer, elk, and moose, is the only prion disease affecting free-ranging animals.

77 ase (CWD) is an emerging and uniformly fatal prion disease affecting free-ranging deer and elk and is

78 s macaque, a highly relevant model for human prion diseases, after a 10-year silent incubation period

79 ty of amyloidogenic proteins associated with prion diseases, Alzheimer's disease, Parkinson's disease

80 tein level, is present in all types of human prion diseases analyzed, although to a different extent

81 from 239 patients with definite or probable prion disease and 100 patients with a definite alternati

82 everal neurodegenerative diseases, including prion disease and Alzheimer disease.

83 ults suggest that A224V is a risk factor for prion disease and modulates the transmission behavior of

84 ays to detect both nonspecific biomarkers of prion disease and prion-specific biomarkers can be used.

85 xpression, occurs at late stages during 263K prion disease and that this dysfunction may be the resul

86 s in neurodegenerative conditions, including prion diseases and Alzheimer's and Parkinson's diseases,

87 lenishment strategies for neuroprotection in prion diseases and possibly other protein misfolding neu

88 gue are potential new therapeutic agents for prion diseases and possibly protein misfolding disorders

89 Recent work with prion diseases and synucleinopathies indicates that accu

90 f screening models that faithfully replicate prion diseases and the lack of rapid, sensitive biologic

91 munity, cancer, neurodegenerative disorders, prion diseases and thrombosis.

92 e.g., to reduce transmission risk related to prion diseases) and the study of protein misfolding; in

93 c wasting disease (CWD), a contagious, fatal prion disease, and compared allele frequency to populati

94 o efficient measures to control this form of prion disease, and, importantly, the zoonotic potential

95 se are called prion strains, or variants, in prion diseases, and cause variation in disease pathogene

96 sition diseases such as Alzheimer's disease, prion diseases, and type II diabetes.

97 Inherited forms of prion disease are caused by mutation of PRNP, whereas ac

98 gest that reports of anticipation in genetic prion disease are driven entirely by ascertainment bias.

99 The prion diseases are a family of fatal neurodegenerative d

100 ssible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative di

101 Prion diseases are a group of fatal neurodegenerative di

102 Prion diseases are a group of incurable neurodegenerativ

103 Mammalian prion diseases are a group of neurodegenerative conditio

104 Prion diseases are a group of progressive and fatal neur

105 Prion diseases are a group of rapidly progressive and al

106 Prion diseases are a group of transmissible, fatal neuro

107 Prion diseases are a unique, infectious, neurodegenerati

108 blish host infection.IMPORTANCE Many natural prion diseases are acquired by oral consumption of conta

109 Many natural prion diseases are acquired orally, and following exposu

110 Many natural prion diseases are acquired orally.

111 Prion diseases are caused by a structural rearrangement

112 Inherited prion diseases are caused by autosomal dominant coding m

113 Prion diseases are caused by PrP(Sc), a self-replicating

114 Prion diseases are caused by the conversion of physiolog

115 Prion diseases are characterized by accumulation of misf

116 cquired and sporadically occurring mammalian prion diseases are controlled by powerful genetic risk a

117 Prion diseases are devastating neurodegenerative disorde

118 Prion diseases are fatal infectious neurodegenerative di

119 Prion diseases are fatal infectious neurodegenerative di

120 Prion diseases are fatal neurodegenerative disorders aff

121 Prion diseases are fatal neurodegenerative disorders cau

122 Prion diseases are fatal neurodegenerative disorders for

123 Prion diseases are fatal neurodegenerative disorders wit

124 Prion diseases are fatal neurodegenerative disorders, wh

125 Prion diseases are infectious neurodegenerative disorder

126 Prion diseases are neurodegenerative disorders pathogeni

127 iseases such as Alzheimer's, Parkinson's and prion diseases are poorly understood.

128 Human prion diseases are rare and usually rapidly fatal neurod

129 The prion diseases are rare neurodegenerative conditions tha

130 Prion diseases are rare, neurological disorders caused b

131 While most prion diseases are species specific, this finding is not

132 Prion diseases are transmissible and fatal neurodegenera

133 Some prion diseases are transmitted, in part, through environ

134 Prion diseases are universally fatal and often rapidly p

135 Prion disease arises upon misfolding of the normal cellu

136 hetic PrP amyloids with or without the human prion disease-associated P102L mutation.

137 entially toxic host response contributing to prion disease-associated pathology.

138 Prion disease-associated retinal degeneration is attribu

139 a novel experimental strategy for preventing prion disease based on producing a self-replicating, but

140 tion has been one of the major challenges in prion disease biology.

141 een purified fH and prion rods enriched from prion-diseased brain.

142 r the detection of multiple human and animal prion diseases but not BSE.

143 into pathogenic PrP conformers is central to prion disease, but the mechanism remains unclear.

144 ing in neurodegenerative diseases, including prion diseases, but the mechanisms facilitating gliosis

145 absolute, protection against transmission of prion disease by blood transfusion.

146 teopathic strains gleaned from the classical prion diseases can be profitably incorporated into resea

147 Phenotypic diversity in prion diseases can be specified by prion strains in whic

148 for understanding cross-seeding specificity.Prion diseases can be transmitted across species.

149 Human prion diseases can have acquired, sporadic, or genetic o

150 About 15% of prion disease cases are genetic, creating an opportunity

151 ne (PRNP) and account for about 15% of human prion disease cases worldwide.

152 ive dementia and accounting for 85% of human prion disease cases, sporadic Creutzfeldt-Jakob disease

153 S) with the P102L mutation is a rare genetic prion disease caused by a pathogenic mutation at codon 1

154 d samples from national blood collection and prion disease centers in the United States and United Ki

155 noculum, demonstrating that GSS is a genuine prion disease characterized by both transmissibility and

156 As the universe of prion diseases continues to expand, mouse models will re

157 oreover, studies on the role of microglia in prion disease could deepen our understanding of neuroinf

158 vious studies established that transmissible prion diseases could be induced by in vitro-produced rec

159 A definitive pre-mortem diagnosis of prion disease depends on brain biopsy for prion detectio

160 Natural transmission of prion diseases depends upon the spread of prions from th

161 ative diseases (n = 352), patients in whom a prion disease diagnosis was likely (n = 105), and patien

162 ng National Prion Clinic referrals in whom a prion disease diagnosis was likely, 2 patients with spor

163 ion during the epidemic of kuru--an acquired prion disease epidemic of the Fore population in Papua N

164 tive diseases like Alzheimer's, Parkinson's, prion diseases, etc.

165 be efficacious in multiple animal models of prion disease even as they revealed new challenges for t

166 Transmissible prion diseases exhibit a spectrum of disease phenotypes

167 Although incurable, prion disease follows a clear pathogenic mechanism, in w

168 Natural forms of prion diseases frequently originate by oral (p.o.) infec

169 ation of a series of 116 patients with other prion diseases from a prospective observational cohort s

170 The genetic prion disease Gerstmann-Straussler-Scheinker syndrome ca

171 trast, three patients referred with possible prion disease had a clinical picture in keeping with aut

172 Unlike other species, prion disease has never been described in dogs even thou

173 tion between structure and susceptibility to prion disease has previously been described.

174 Previous trials in prion disease have been done in symptomatic patients who

175 studies in which more than 300 patients with prion disease have been followed up from diagnosis to de

176 as sporadic, genetic, and acquired forms of prion disease have different clinical and laboratory pre

177 etting, and consequently used to treat human prion diseases, improves replicative ability in another

178 rol and Prevention's diagnostic criteria for prion disease in 2018.

179 of 263K in vitro; however, it did not delay prion disease in animals.

180 ible for approximately 10 to 15% of cases of prion disease in humans, including Creutzfeldt-Jakob dis

181 ectly mirror those generated in an inherited prion disease in humans.

182 ormers at the earliest stages of preclinical prion disease in mice and precedes the maximum infectiou

183 cal landmarks to describe the progression of prion disease in vivo.

184 pecifically, we established a mouse model of prion disease in which the 79A murine prion strain was i

185 g support for the protein-only hypothesis of prion diseases in its pure form, arguing against the not

186 called prions are associated with infectious prion diseases in mammals and inherited phenotypes in ye

187 A candidate biomarkers could be selected for prion diseases in multiple species.

188 To minimize further spreading of prion diseases in small ruminants the development of a h

189 It is unique among prion diseases in that it is transmitted naturally throu

190 After exposure, the accumulation of some prion diseases in the gut-associated lymphoid tissues (G

191 Several complement proteins exacerbate prion disease, including C3, C1q, and CD21/35.

192 reductions in PrPC occur in a wide range of prion diseases, including sheep scrapie, human Creutzfel

193 ory loss and slowed the progression of mouse prion disease, indicating that this ligand type may have

194 el the PRNP A117V mutation causing inherited prion disease (IPD) including Gerstmann-Straussler-Schei

195 Prion disease is a rapidly progressive neurodegenerative

196 Prion disease is a rare, fatal, and exceptionally rapid

197 s signaling exclusively in astrocytes during prion disease is alone sufficient to prevent neuronal lo

198 the dominance of subfibrillar aggregates in prion disease is due to the replication of GPI-anchored

199 A common presentation of inherited prion disease is Gerstmann-Straussler-Scheinker syndrome

200 evidence to guide the care of patients with prion disease is scarce.

201 Although the unifying hallmark of prion diseases is CNS neurodegeneration caused by confor

202 One of the most puzzling aspects of the prion diseases is the intricate relationship between pri

203 The wide phenotypic variability of prion diseases is thought to depend on the interaction o

204 hich is the key event in the pathogenesis of prion diseases, is indicative of a conformationally flex

205 Prion diseases, like Alzheimer's disease and Parkinson d

206 Thus, in prion-diseased mice, both trazodone and dibenzoylmethane

207 f protein aggregation, which is the basis of prion disease, might underlie the progression of patholo

208 ne cell population of the brain, in a murine prion disease model of chronic neurodegeneration.

209 nt immune cell population of the brain, in a prion disease model of chronic neurodegeneration.

210 erative diseases (n = 34), and patients with prion disease (n = 37) of which 20 had sCJD.

211 Chronic wasting disease (CWD) is the only prion disease naturally transmitted among farmed and fre

212 e regulatory epitranscriptomic mechanisms in prion disease neuropathogenesis, whereby RNA-editing tar

213 The prion diseases occur following the conversion of the cel

214 In humans, prion disease occurs typically with a sporadic origin wh

215 reutzfeldt-Jakob disease-the human form of a prion disease of cattle, bovine spongiform encephalopath

216 wasting disease (CWD) is a rapidly spreading prion disease of cervids, yet antemortem diagnosis, trea

217 ease (CWD) is an emergent, rapidly spreading prion disease of cervids.

218 Chronic wasting disease (CWD) is a fatal prion disease of North American deer and elk and poses a

219 Atypical/Nor98 scrapie (AS) is a prion disease of small ruminants.

220 Prion diseases of cattle include the classical bovine sp

221 The patients had either inherited prion disease or sporadic Creutzfeldt-Jakob disease.

222 on formation in sporadic and inherited human prion diseases or equivalent animal diseases are poorly

223 an be used to measure disease progression in prion diseases or predict disease onset in healthy indiv

224 Prion diseases, or transmissible spongiform encephalopat

225 The role of the GPI-anchor in prion disease pathogenesis is still a challenging issue.

226 estinal infection would similarly affect CNS prion disease pathogenesis.

227 e 2-polarized immune response did not affect prion disease pathogenesis.

228 a helminth co-infection would influence oral prion disease pathogenesis.

229 may be important factors that influence oral prion disease pathogenesis.

230 I of 1,458 patients referred to the National Prion Disease Pathology Surveillance Center were collect

231 g patients with iCJD, in contrast with other prion disease patients and population controls, is consi

232 eutzfeldt-Jakob disease and genetic forms of prion disease), patients with other degenerative or nond

233 highlight PTMs as a major force driving the prion disease phenotype.

234 Understanding how host pathways can modify prion disease phenotypes may provide clues on how to alt

235 signaling is maintained in both AD and mouse prion disease points to the latter as an excellent model

236 n in 5XFAD mice and throughout the course of prion disease, preventing behavioural deficits and neuro

237 on of the importance of microglia within the prion disease process and identifies the nature of the r

238 g stress-induced HSP70 exhibited accelerated prion disease progression compared with WT mice.

239 an important role in suppressing or delaying prion disease progression, opening opportunities for the

240 ant prion protein deposition and accelerated prion disease progression.

241 Recently, we showed that in prion disease, protein misfolding leads to neurodegenera

242 loprotease ADAM10, yet the impact of this on prion disease remains enigmatic.

243 Prion diseases represent the archetype of brain diseases

244 Prion diseases represent the archetype of brain diseases

245 d in the pathogenesis and the progression of prion diseases, representing a valid tool for distinguis

246 rm encephalopathy (c-BSE) is the only animal prion disease reputed to be zoonotic, causing variant Cr

247 Prion diseases require host expression of the prion prot

248 el that replicates human prions has hampered prion disease research for decades.

249 long-standing gap in the repertoire of human prion disease research, providing a new in vitro system

250 ediate CSF RT-QuIC patterns, whereas genetic prion diseases revealed distinct profiles for each PRNP

251 er's (AD), Parkinson's (PD), Huntington's or Prion diseases share similar pathological features.

252 c Creutzfeldt-Jakob disease; kuru; inherited prion disease; sheep scrapie; bovine spongiform encephal

253 s from sporadic CJD, the most common form of prion disease, showed the highest sensitivity.

254 Here, we have shown that mouse prion disease shows many of the hallmarks of human AD, i

255 d with a Gerstmann-Straussler-Scheinker-like prion disease) spontaneously forms amyloid fibrils with

256 IC is superior to surrogate marker tests for prion diseases such as 14-3-3 and tau proteins, and toge

257 Human prion diseases such as Creutzfeldt-Jakob disease are tra

258 on protein (PrP) has been implicated both in prion diseases such as Creutzfeldt-Jakob disease, where

259 rying mutations analogous to human heritable prion diseases, support that mutations might predispose

260 Our data show that oral prion disease susceptibility was dramatically reduced in

261 icular dendritic cells was impaired and oral prion disease susceptibility was reduced.

262 cal event in neurodegenerative diseases like prion diseases, synucleinopathies, and tauopathies that

263 obust genetic associations in sporadic human prion disease that implicate intracellular trafficking a

264 pongiform encephalopathy (BSE) in cattle are prion diseases that are caused by the same protein-misfo

265 generate recombinant versions of other human prion diseases that could provide a further understandin

266 esults imply that OSCAR is a robust model of prion diseases that offers a promising platform for unde

267 Our results suggest that prion diseases that produce higher levels of anchorless

268 In most human sporadic prion diseases the phenotype is consistently associated

269 uIC) to model the central molecular event in prion disease, the templated misfolding of the normal pr

270 IMPORTANCE In prion diseases, the prion protein misfolds and aggregate

271 n certain sporadic, familial, and infectious prion diseases, the prion protein misfolds and aggregate

272 the CNS, and will facilitate development of prion disease therapeutics with this mechanism of action

273 Using a scrapie mouse model of prion disease to assess various time points postinoculat

274 Prion diseases (transmissible spongiform encephalopathie

275 input for the risk assessment of blood-borne prion disease transmission and for refining the target p

276 input for the risk assessment of blood-borne prion disease transmission and for refining the target p

277 plies in order to mitigate the risk of human prion disease transmission.

278 variant Creutzfeldt-Jakob disease (vCJD), a prion disease typically acquired from consumption of pri

279 ssible for swine to serve as a reservoir for prion disease under natural conditions.

280 dropouts) with early to moderately advanced prion disease using model parameters to compare the powe

281 Here, we studied the role of GRP78 in prion diseases using several in vivo and in vitro approa

282 The assay's specificity for prion disease was 100% (95% CI, 97%-100%), with no false

283 Increased abundance of ABCA1 in prion disease was confirmed in prion-infected mice.

284 owledge of the neuroinflammatory response in prion diseases, we assessed the expression of key genes

285 itical for toxic signaling by AbetaOs and in prion diseases, we tested whether mGlur5 knock-out mice

286 The memory deficits we observed in mouse prion disease were completely restored by treatment with

287 he nervous system and critically involved in prion diseases where it misfolds into pathogenic PrP(Sc)

288 an extensive reanalysis of a large study of prion disease, where the transcriptome of mouse brains h

289 th brain- or PMCAb-derived PrP(Sc) developed prion disease, whereas administration of dsPMCAb-derived

290 plays an important role in the diagnosis of prion disease, which is often challenging to diagnose.

291 Among these disorders are the prion diseases, which are transmissible, and in which th

292 rences in pathogenesis and pathology between prion diseases, which uniquely involve aggregation of a

293 ause fatal disease, as with human iatrogenic prion diseases, while other aggregates appear to be rela

294 4) version of these fibrils develop clinical prion disease with a 100% attack rate.

295 etter assess the zoonotic potential of other prion diseases with high prevalence, notably Chronic Was

296 uantitative EEG to follow the progression of prion disease, with potential to help evaluate the treat

297 own to increase survival in animal models of prion disease, with proposed mechanisms including calcin

298 ongly up-regulated in the brain of all human prion diseases, with only a mild up-regulation in AD.

299 with chronic neurodegenerative disease (ME7 prion disease) would display exaggerated responses to ce

300 What makes one animal prion disease zoonotic and others not is poorly understo