ライフサイエンスコーパス: prion disease

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

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

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

4 hlight the role of proteolytic processing in prion disease.

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

6 ransition from presymptomatic to symptomatic prion disease.

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

8 f neuropathological findings associated with prion disease.

9 is invertebrate host as a model of mammalian prion disease.

10 idered when treating patients with suspected prion disease.

11 of Alzheimer disease, is modeled well in ME7 prion disease.

12 tility of Drosophila as a model of mammalian prion disease.

13 a particular neurodegenerative phenotype or prion disease.

14 al of primary progressive aphasia to include prion disease.

15 oglial proliferation, using a mouse model of prion disease.

16 isease (sCJD), the most common form of human prion disease.

17 st does not always result in a transmissible prion disease.

18 gnificance in the meta-analysis of all human prion disease.

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

20 n many neurodegenerative diseases, including prion disease.

21 itochondrial processes may be altered during prion disease.

22 me, specifically discriminates patients with prion disease.

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

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

25 oped clinical neurologic signs suggestive of prion disease.

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

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

28 utic implications through the examination of prion disease.

29 ent of ultrasensitive methods for diagnosing prion disease.

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

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

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

33 t constitute a novel therapeutic approach to prion diseases.

34 tial therapeutic target for the treatment of prion diseases.

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

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

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

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

39 cies transmission barriers characteristic of prion diseases.

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

41 scrapie PrP (PrP(Sc)) is a central event in prion diseases.

42 ed our understanding of sporadic and genetic prion diseases.

43 atients and for prophylactic use in familial prion diseases.

44 ateral sclerosis, cerebral ischemia, and the prion diseases.

45 (atypical PrPres) were recently described in prion diseases.

46 nditions such as Alzheimer's, Parkinson, and prion diseases.

47 s agents responsible for the transmission of prion diseases.

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

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

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

51 milar mechanism by which prions propagate in prion diseases.

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

53 incurable diseases including Alzheimer's and prion diseases.

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

55 ansmissible proteinopathies rather than true prion diseases.

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

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

58 isorders, and is particularly conspicuous in prion diseases.

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

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

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

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

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

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

65 design of diagnostic assays that can detect prion disease across the diversity of sporadic CJD subty

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

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

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

69 ical step in the pathogenesis of amyloid and prion diseases, although the molecular mechanisms underl

70 Human prion diseases, although variable in clinicopathological

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

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

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

74 neurodegeneration in the ME7 mouse model of prion disease and by superimposing systemic inflammation

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

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

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

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

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

80 between individuals were first described in prion diseases and proposed as the basis of their infect

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

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

83 line, slow linear decline (usually inherited prion disease) and in some patients, decline followed by

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

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

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

87 clerosis, amyotrophic lateral sclerosis, the prion diseases, and familial encephalopathy with neurose

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

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

90 Approximately 15% of cases of recognized prion disease are inherited and associated with coding m

91 The prion diseases are a family of fatal neurodegenerative d

92 Prion diseases are a group of fatal neurodegenerative di

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

94 Prion diseases are a group of progressive and fatal neur

95 Prion diseases are a group of transmissible, fatal neuro

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

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

98 Many natural prion diseases are acquired orally.

99 ssible spongiform encephalopathies (TSEs) or prion diseases are associated with accumulations of dise

100 Prion diseases are associated with the conformational co

101 Prion diseases are caused by a structural rearrangement

102 Prion diseases are caused by the structural conversion o

103 Prion diseases are characterized by a conformational cha

104 Prion diseases are characterized by accumulation of misf

105 Prion diseases are characterized by the conversion of th

106 Prion diseases are characterized by tissue accumulation

107 Prion diseases are devastating neurodegenerative disorde

108 Prion diseases are fatal infectious neurodegenerative di

109 Prion diseases are fatal neurodegenerative disorders aff

110 Prion diseases are fatal neurodegenerative disorders ass

111 Prion diseases are fatal neurodegenerative disorders for

112 Prion diseases are fatal neurodegenerative disorders wit

113 Prion diseases are fatal neurodegenerative disorders, wh

114 Prion diseases are infectious neurodegenerative disorder

115 Prion diseases are neurodegenerative disorders character

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

117 Prion diseases are rare fatal neurological conditions of

118 The prion diseases are rare neurodegenerative conditions tha

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

120 Prion diseases are transmissible and fatal neurodegenera

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

122 Prion diseases are universally fatal and often rapidly p

123 encephalopathies, more commonly known as the prion diseases, are associated with the production and a

124 Prion disease arises upon misfolding of the normal cellu

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

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

127 Prion disease-associated retinal degeneration is attribu

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

129 synucleinopathies, hippocampal sclerosis and prion disease) based on a neuropathological examination

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

131 Alzheimer and prion disease brain tissues did not do so, demonstrating

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

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

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

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

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

137 Prion disease can be initiated in animal models by inocu

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

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

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

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

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

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

144 sible spongiform encephalopathies (TSEs), or prion diseases, contain as a major component PrP(Sc), an

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

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

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

148 al distinction between the invariably lethal prion disease Creutzfeldt-Jakob disease (CJD) and nonpri

149 es in muscle of transgenic mice that develop prion disease de novo.

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

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

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

153 strates that a new mechanism responsible for prion diseases different from the PrP(Sc)-templated or s

154 hronic wasting disease (CWD), the only known prion disease endemic in wildlife, is a persistent probl

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

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

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

158 Transmissible prion diseases exhibit a spectrum of disease phenotypes

159 to a previous knock-in model of a different prion disease [fatal familial insomnia (FFI)].

160 ary between sporadic, acquired and inherited prion diseases following clinical expectations.

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

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

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

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

165 However, a transgenic mouse model of prion disease has demonstrated that prion infectivity ca

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

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

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

169 animal transmission, to characterize a novel prion disease in a large British kindred.

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

171 halopathy (BSE) epidemic, when >200 cases of prion disease in humans were diagnosed as variant Creutz

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

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

174 en shown to significantly delay the onset of prion disease in mice, and humanized versions are candid

175 IVF) performed for the prevention of genetic prion disease in the children of a 27-year-old asymptoma

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

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

178 rP-res(17kDa) was highly infectious, causing prion disease in wild-type mice with an average survival

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

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

181 a sound basis for risk assessment regarding prion diseases in purportedly resistant species.

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

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

184 r findings suggest a new avenue for treating prion diseases, in which a patient's own unglycosylated

185 Molecular markers of prion disease include accumulation of the misfolded prio

186 culated cats developed signs consistent with prion disease, including a stilted gait, weight loss, an

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

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

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

190 In most forms of prion disease, infectivity is present primarily in the c

191 since neuronal antibodies may be positive in prion disease, interpretation can be complex and must be

192 Prion diseases involve the conformational conversion of

193 ress in therapeutics for rare disorders like prion disease is impeded by the lack of validated outcom

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

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

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

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

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

199 Conversely, clinical manifestations of prion disease may occur either before or in the absence

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

201 icity and significantly reducing survival in prion-diseased mice.

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

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

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

205 since Mte successfully rescued the mice from prion disease, Mte might be used for remediation and dec

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

207 The prion diseases occur following the conversion of the cel

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

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

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

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

212 PrP(res) from sheep affected by scrapie, the prion disease of small ruminants, to rapidly assess the

213 itive prionopathy is a newly described human prion disease of unknown aetiology lying out with the hi

214 Prion diseases of cattle include the classical bovine sp

215 Prion diseases of deer and sheep can be transmitted via

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

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

218 Prion diseases, or transmissible spongiform encephalopat

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

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

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

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

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

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

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

226 s of PrP may be of crucial importance in the prion disease process.

227 ant prion protein deposition and accelerated prion disease progression.

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

229 e proposed 20-point Medical Research Council prion disease rating scale assesses domains of cognitive

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

231 s and PrP(Sc), and their role in etiology of prion diseases, remains unknown.

232 Prion diseases represent the archetype of brain diseases

233 Prion diseases represent the archetype of brain diseases

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

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

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

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

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

239 e and propose that future clinical trials in prion disease should collect data compatible with this s

240 e to tau and TAR DNA-binding protein 43, and prion disease showed a lower prevalence of coincident ce

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

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

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

244 frequently co-exist in the most common human prion disease, sporadic Creutzfeldt-Jakob disease.

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

246 Prion diseases such as Creutzfeldt-Jakob disease (CJD) a

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

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

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

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

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

252 of host PrP is the major determinant of host prion disease susceptibility.

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

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

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

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

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

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

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

260 e disorders such as Alzheimer, Parkinson, or prion diseases, the conversion of soluble proteins into

261 Importance: During prion diseases, the host protein PrPC converts into an a

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

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

264 ent levels equivalent to that of other human prion diseases, these data indicate that variably protea

265 ting that it can influence susceptibility to prion disease through two distinct mechanisms.

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

267 Outside of scrapie-mediated prion disease, to our knowledge, this findiing is the fi

268 Prion diseases (transmissible spongiform encephalopathie

269 animal mortalities potentially infected with prion diseases (transmissible spongiform encephalopathie

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

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

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

273 ng disorders, including Alzheimer's disease, prion diseases, type II diabetes, and others.

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

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

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

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

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

279 rogressive chronic neurodegeneration, murine prion disease, we define the temporal dynamics of the ge

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

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

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

283 irculating levels of Clusterin in late-stage prion disease when animals will show behavioral decline,

284 receptors CD21/35 partially resist terminal prion disease when infected i.p. with mouse-adapted scra

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

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

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

288 eased neurogenesis during the progression of prion disease, which partially counteracts the effects o

289 prionopathy is a rare phenotype within human prion diseases, which are themselves rare.

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

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

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

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

294 llular prions PrP(C) as a model biomarker of prion disease with high sensitivity.

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