ライフサイエンスコーパス: CJD

1 CJD-infected microglia also displayed morphological chan

2 was found in 89% of tested CJD cases and 0% CJD mimics.

3 1), possible (n = 24), or suspected (n = 11) CJD and 104 negative control samples (54 CSF and 50 OM).

4 est to classify cases correctly (92% CJD, 2% CJD mimics).

5 10-24 brain regions (100%) examined from 28 CJD patients.

6 le tissue specimens taken at autopsy from 49 CJD patients in the United Kingdom.

7 trations showed similar profiles among the 7 CJD types analyzed, PrP(Sc) exposure to increasing tempe

8 ilable test to classify cases correctly (92% CJD, 2% CJD mimics).

9 sion and reduces seizure susceptibility in a CJD mouse model.

10 eported C-terminal PrP mutation (A224V) in a CJD patient exhibiting a disease similar to the rare VV1

11 contribute to neuroinvasion, we inoculated a CJD agent into mutant mice that (i) lacked B cells, (ii)

12 and from those seen in sporadic and acquired CJD.

13 s account for only a small proportion of all CJD cases.

14 The test discriminated between control and CJD-infected brains.

15 ssociated with Parkinson's, Alzheimer's, and CJD diseases.

16 atypical AD phenotypes wrongly classified as CJD.

17 Creutzfeldt-Jakob disease (CJD), as well as CJD-affected subjects who might have been exposed to CWD

18 milar age, sex and frequency of dementia but CJD mimics had a longer clinical history.

19 atic patients with various mutations causing CJD or Gerstmann-Straussler-Scheinker syndrome, 6 had po

20 es its relative protection against classical CJD and kuru in the heterozygous state.

21 concentration among pathologically confirmed CJD patients (28.0+/-20.6 ng/ml, n = 41) is significantl

22 In contrast, CJD mice had the hallmark features of CJD, spongiosis an

23 We specifically tested a previously defined CJD biomarker profile (T-tau >1400 ng/L and T-tau to P-t

24 All definite CJD cases were comprehensively tested for NSA-abs, with

25 contrast, none of 49 patients with definite CJD had NSA-abs, including NMDAR-abs, GlyR-abs, LGI1-abs

26 None of the 49 patients with definite CJD had NSA-abs.

27 suspected CJD and 49 patients with definite CJD.

28 ive (suspected) and retrospective (definite) CJD cohort study was conducted in a reference center for

29 erinfection by a more virulent human-derived CJD agent (FU-CJD) and does not require pathological pri

30 be considered and excluded before diagnosing CJD.

31 riod who had an alternative final diagnosis (CJD mimics).

32 in mice and rats infected with two different CJD strains, but not in CJD-infected hamsters.

33 ) controls with a Creutzfeldt-Jakob disease (CJD) agent.

34 ened awareness of Creutzfeldt-Jakob disease (CJD) among physicians and the lay public has led to its

35 from 60 sporadic Creutzfeldt-Jakob disease (CJD) and 6 variant CJD brains.

36 Creutzfeldt-Jakob disease (CJD) and autoimmune encephalitis with antibodies against

37 heimer's disease, Creutzfeldt-Jakob disease (CJD) and Gerstmann-Straussler-Scheinker syndrome have be

38 hal prion disease Creutzfeldt-Jakob disease (CJD) and nonprion rapidly progressive dementias is impor

39 a central role in Creutzfeldt-Jakob Disease (CJD) and other transmissible spongiform encephalopathies

40 Human Creutzfeldt-Jakob disease (CJD) and similar neurodegenerative diseases such as shee

41 and Kuru disease, Creutzfeldt-Jakob disease (CJD) and variant CJD in humans.

42 disease (AD) and Creutzfeldt-Jakob disease (CJD) are clinically distinguishable, atypical AD phenoty

43 F) biomarkers for Creutzfeldt-Jakob disease (CJD) are established and partly included in the diagnost

44 diseases such as Creutzfeldt-Jakob disease (CJD) are fatal, neuro-degenerative disorders with no kno

45 diseases such as Creutzfeldt-Jakob disease (CJD) are incurable and rapidly fatal neurodegenerative d

46 nts with sporadic Creutzfeldt-Jakob disease (CJD) bind to very low-density (VLDL) and low-density (LD

47 rated that PrP in Creutzfeldt-Jakob disease (CJD) brains is cleaved by a cellular protease to generat

48 umber of sporadic Creutzfeldt-Jakob disease (CJD) cases.

49 d anticipation in Creutzfeldt-Jakob disease (CJD) caused by the c.598G > A mutation in PRNP encoding

50 humans as variant Creutzfeldt-Jakob disease (CJD) has affected over 100 individuals, and probably mil

51 Creutzfeldt-Jakob disease (CJD) has been accidentally transmitted by contaminated c

52 l transmission of Creutzfeldt-Jakob disease (CJD) has been demonstrated, these iatrogenic cases accou

53 evious studies in Creutzfeldt-Jakob disease (CJD) have shown that myeloid cells in the periphery as w

54 ions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE

55 ses which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy and

56 Creutzfeldt-Jakob disease (CJD) in humans has been shown to be transmissible via se

57 sorders including Creutzfeldt-Jakob disease (CJD) in humans, is the conversion of the normal or cellu

58 ie in animals and Creutzfeldt-Jakob disease (CJD) in humans.

59 e and PrP(CJD) in Creutzfeldt-Jakob disease (CJD) in humans.

60 Importance: Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder associated wi

61 Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder caused by prion pro

62 Creutzfeldt-Jakob disease (CJD) is a rare but invariably fatal neurodegenerative di

63 Creutzfeldt-Jakob disease (CJD) is a rare progressive neurodegenerative disorder, c

64 vivo diagnosis of Creutzfeldt-Jakob disease (CJD) is necessary for quickly distinguishing treatable f

65 Sporadic Creutzfeldt-Jakob disease (CJD) is the most prevalent manifestation of the transmis

66 Variant Creutzfeldt-Jakob disease (CJD) is thought to be caused by dietary or other exposur

67 ropagated various Creutzfeldt-Jakob disease (CJD) isolates (sporadic, variant, and iatrogenic CJD) in

68 g patients with a Creutzfeldt-Jakob disease (CJD) phenotype.

69 uru and classical Creutzfeldt-Jakob disease (CJD) prions (which are closely similar) but can be infec

70 ation periods for Creutzfeldt-Jakob disease (CJD) prions than mice expressing full-length human PrP.

71 viduals developed Creutzfeldt-Jakob disease (CJD) worldwide as a result of treatment, typically in ch

72 eneration (FTLD), Creutzfeldt-Jakob disease (CJD), Alzheimer's disease (AD), Parkinson's disease (PD)

73 individuals with Creutzfeldt-Jakob disease (CJD), an incurable brain disorder caused by alterations

74 cts with sporadic Creutzfeldt-Jakob disease (CJD), as well as CJD-affected subjects who might have be

75 iseases including Creutzfeldt-Jakob disease (CJD), but the aggregation mechanisms remain poorly under

76 isorder, sporadic Creutzfeldt-Jakob disease (CJD), in which prions are formed spontaneously from wild

77 ople as a variant Creutzfeldt-Jakob disease (CJD), it becomes critical to identify cells in the perip

78 us agents causing Creutzfeldt-Jakob disease (CJD), scrapie, and bovine spongiform encephalopathy beca

79 of transmissible Creutzfeldt-Jakob disease (CJD), the ataxia of Tg(A116V) mice is more prominent, an

80 Creutzfeldt-Jakob disease (CJD), the first transmissible spongiform encephalopathy

81 Creutzfeldt-Jakob disease (CJD), the most common human prion disease, includes spor

82 ally diagnosed as Creutzfeldt-Jakob disease (CJD).

83 sample of variant Creutzfeldt-Jakob disease (CJD).

84 man with sporadic Creutzfeldt-Jakob disease (CJD).

85 prion disease is Creutzfeldt-Jakob disease (CJD).

86 plied to diagnose Creutzfeldt-Jakob disease (CJD).

87 diseases such as Creutzfeldt-Jakob disease (CJD).

88 l fluid marker of Creutzfeldt-Jakob disease (CJD).

89 rodent models of Creutzfeldt-Jakob disease (CJD).

90 humans, including Creutzfeldt-Jakob disease (CJD).

91 re human disorder Creutzfeldt-Jakob disease (CJD).

92 mans with variant Creutzfeldt-Jakob disease (CJD); on second passage in Tg(BoPrP) mice, the incubatio

93 ilial or sporadic Creutzfeldt-Jakob disease (CJD); there was no case of variant CJD.

94 f these diseases [Creutzfeldt-Jakob disease (CJD)] that was exactly analogous to a previous knock-in

95 TD/ALS, n = 252), Creutzfeldt-Jakob disease (CJD, n = 239), Parkinson's disease (PD, n = 39), dementi

96 (AD p<0.05, PD p<0.01, FTLD p<0.0001) except CJD.

97 By rapidly confirming or excluding CJD with high accuracy the assay is expected to improve

98 n = 11, sporadic CJD (sCJD) n = 23, familial CJD (gCJD) n = 17, fatal familial insomnia (FFI) n = 9,

99 ssues of patients with sporadic and familial CJD.

100 aditionally have been classified as familial CJD, Gerstmann-Straussler-Scheinker syndrome, or fatal f

101 Prevalence of the 200K variant of familial CJD is especially high in Slovakia, Chile, and Italy, an

102 ional octapeptide repeats linked to familial CJD.

103 In mouse brain, the fast CJD strain, FU, elicits many PrP-res deposits, whereas t

104 Adherence to revised criteria for CJD, which include distinctive magnetic resonance imagin

105 ection (e.g. country of origin, deferral for CJD risk factors) currently occupies the front line for

106 we review recent lead discovery efforts for CJD, with a focus on our own efforts to optimize 2-amino

107 on of the PRNP gene may be a risk factor for CJD.

108 ble disorders are most commonly mistaken for CJD.

109 iven the absence of any treatment option for CJD patients and the favorable drug characteristics of a

110 In summary, FDC are not required for CJD agent spread from the periphery, although FDC may en

111 Eye donors are not ordinarily tested for CJD, in part because an easy test is not available.

112 barrier, it may be considered as therapy for CJD patients and for prophylactic use in familial prion

113 may offer a novel symptomatic treatment for CJD.SIGNIFICANCE STATEMENT Dementia and myoclonic jerks

114 In the second case, EEG was not typical for CJD but the clinical course of the disease confirmed tha

115 Transcript profiles unique for CJD microglia and other myeloid cells provide opportunit

116 eatures of GSS(A117V) that are distinct from CJD, supporting PrP(A116V) to carry specific phenotypic

117 to ascertain possible prion infectivity from CJD in the amniotic fluid.

118 Microglia purified from CJD-infected mice showed infectivity comparable to that

119 a more virulent human-derived CJD agent (FU-CJD) and does not require pathological prion protein (Pr

120 However, only 22L and not Ch prevented FU-CJD infection, even though both scrapie strains provoked

121 nversion (RT-QuIC)) for sporadic and genetic CJD.

122 urrent geographic distribution of hereditary CJD associated with the 200K mutation.

123 ene is the most frequent cause of hereditary CJD, accounting for >70% of families with CJD worldwide.

124 derived from human iPSCs can replicate human CJD prions.

125 gh such treatment ceased in 1985, iatrogenic CJD (iCJD) continues to emerge because of the prolonged

126 eldt-Jakob disease (vCJD) n = 20, iatrogenic CJD (iCJD) n = 11, sporadic CJD (sCJD) n = 23, familial

127 isolates (sporadic, variant, and iatrogenic CJD) in neuronal cultures expressing the human prion pro

128 eptible to sporadic, familial, or iatrogenic CJD prions.

129 c CJD (sCJD), variant CJD (vCJD), iatrogenic CJD, inherited prion disease, kuru and resistance to kur

130 n a small sample of patients with iatrogenic CJD (p=0.030) but not in patients with sporadic CJD (sCJ

131 logical diagnostic biomarker for identifying CJD, 14-3-3 protein in cerebrospinal fluid (CSF), unfort

132 In CJD individuals (n = 30) with repeated measurements, but

133 The finding of PrP-CTF12/13 in CJD brains widens the heterogeneity of the PK-resistant

134 ented, illustrating further heterogeneity in CJD, and suggesting that further molecular subtypes of C

135 Both NF-L and tau were markedly increased in CJD serum, reaching similar or even better performance a

136 d with two different CJD strains, but not in CJD-infected hamsters.

137 13 kDa midspan PrP fragment, not observed in CJD.

138 evels and increased T-tau to P-tau ratios in CJD patients has a very high specificity against importa

139 d the enhanced susceptibility to seizures in CJD and raise the possibility that targeting IL-1beta wi

140 out how best to intervene therapeutically in CJD, prion infections can be propagated in cell culture

141 In the absence of known CJD risk factors or prion protein gene abnormalities, th

142 most common treatable condition that mimics CJD, we believe that it is crucial to screen all patient

143 ilable from information held at the National CJD Research and Surveillance Unit and includes the dura

144 roven cases of vCJD referred to the National CJD Surveillance Unit (NCJDSU) between 1995 and 2004 and

145 The National CJD Surveillance Unit prospectively identified 84 people

146 The national CJD surveillance unit reported all cases of probable or

147 ed cases of variant CJD through the National CJD Surveillance Unit, and obtained lifetime residential

148 on detection in about 80% of PQ-CSF negative CJD samples.

149 Moreover, even non-CJD deaths exhibit 16 years anticipation (p = 0.002), in

150 ), 28 control brains of individuals with non-CJD neurodegenerative diseases and 10 normal brains.

151 In the overall meta-analysis of CJD, 14 SNPs were associated (P < 10(-5); two at PRNP, t

152 e and modulates the transmission behavior of CJD prions in a strain-specific manner, arguing that res

153 tive and gestational tissues in this case of CJD.

154 finite cases of AD from 52 definite cases of CJD.

155 lgorithm for accurate and early diagnosis of CJD by using the RT-QuIC assay on CSF samples, OM sample

156 en considering the differential diagnosis of CJD compared with atypical AD phenotypes, CSF t-PrP dete

157 The 14-3-3 ELISA supports a diagnosis of CJD in patients who fulfill clinical criteria for possib

158 regarded as sufficient for the diagnosis of CJD.

159 Patients who died of CJD had elevated CSF T-tau levels and T-tau to P-tau rat

160 ared from the brains of patients who died of CJD.

161 reen brains of cornea donors for evidence of CJD.

162 trast, CJD mice had the hallmark features of CJD, spongiosis and proteinase K-resistant PrP aggregate

163 y absent in the more common sporadic form of CJD (sCJD).

164 In 1996, a newly recognized variant form of CJD among young patients (median age, 28 years) with unu

165 variants, which included a thalamic form of CJD and a phenotype characterized by prominent dementia

166 Of concern with the variant form of CJD, unlike the more classic forms of the disease, is th

167 Tg(CJD) mice, which model a genetic form of CJD.

168 f PrP(Sc) in muscle tissue from all forms of CJD indicates the possible presence of infectivity in th

169 re underlie susceptibility to these forms of CJD.

170 To establish the independence of CJD agent characteristics from those of PrP-res, two dif

171 However, the majority of CJD cases are not associated with mutations in the PRNP

172 The continuous substantial replication of CJD in monotypic cells will further the discrimination o

173 replication of prions from brain samples of CJD patients.

174 esent in brains of donors at early stages of CJD.

175 uggesting that further molecular subtypes of CJD may exist at lower frequencies.

176 rapid neurologic deterioration suggestive of CJD.

177 ify atypical AD phenotypes with suspicion of CJD based on a decision tree combining CSF biomarkers.

178 ord of patients with ALS, but not Con, AD or CJD cases, CHIT1 was expressed in the corticospinal trac

179 ose of PrP-res, two different mouse-passaged CJD strains were propagated in neuronal cell lines whose

180 s who fulfill clinical criteria for possible CJD.

181 diagnostic criteria of probable or possible CJD.

182 diagnostic criteria for probable or possible CJD.

183 At present, CJD is an invariably fatal disease with no immediate pro

184 crucial to screen all patients with presumed CJD for this reversible condition.

185 appeared allowed for a diagnosis of probable CJD.

186 iform encephalopathy (BSE) in cattle and PrP(CJD) in Creutzfeldt-Jakob disease (CJD) in humans.

187 plate-based fluorescence assay involving PrP(CJD)-seeded polymerization of recombinant PrP into amylo

188 zfeldt-Jakob disease, the prion protein (PrP(CJD)), by means of real-time quaking-induced conversion

189 VV2, the most prevalent and fast-replicating CJD types, showed stable and highly resistant PrP(Sc) ag

190 a1) were progressively upregulated in rodent CJD, the temporal patterns and peak magnitudes of each o

191 ilar to the rare VV1 subtype of sporadic (s) CJD and investigate the role of this mutation in prion r

192 22372, which was susceptible to sporadic (s) CJD prions in approximately 110 days.

193 Sporadic CJD and BSE agents and representative scrapie agents wer

194 = 20, iatrogenic CJD (iCJD) n = 11, sporadic CJD (sCJD) n = 23, familial CJD (gCJD) n = 17, fatal fam

195 However, the ability of a sporadic CJD molecular subtype to use a specific PrP(C) substrate

196 rs for the diagnosis of genetic and sporadic CJD and might represent promising tools to predict disea

197 The majority are sporadic CJD (sCJD) cases of unknown cause.

198 ncing of these regions in controls, sporadic CJD (sCJD) and variant CJD (vCJD) patients has identifie

199 or additional factors may determine sporadic CJD subtype.

200 rmed the presence of three distinct sporadic CJD molecular subtypes with PrP(C) substrate requirement

201 A proposed diagnostic algorithm for sporadic CJD combines CSF and OM RT-QuIC testing to provide virtu

202 Isolates from sporadic CJD, the most common form of prion disease, showed the h

203 rains from confirmed cases of human sporadic CJD and sampled each in triplicate (18 specimens), 28 co

204 of type 1 human PrP(Sc) present in sporadic CJD affecting subjects homozygous for methionine at codo

205 the "typical" appearances found in sporadic CJD, about half the cases tested had a positive 14-3-3 i

206 th BSE-derived prions may result in sporadic CJD-like or novel phenotypes in addition to vCJD, depend

207 te prions associated with the major sporadic CJD strains found in human patients.

208 We conducted GWAS of sporadic CJD (sCJD), variant CJD (vCJD), iatrogenic CJD, inherite

209 digestive enzyme (DE) treatment of sporadic CJD brain homogenate generates a C-terminal fragment sim

210 A molecular classification of sporadic CJD is proposed.

211 rate of approximately 1.1 cases of sporadic CJD per 1 million people per year in Washington.

212 ntrast, three different isolates of sporadic CJD prions failed to transmit disease to Tg(GPPrP) mice.

213 ion disease across the diversity of sporadic CJD subtypes.

214 t is indistinguishable from that of sporadic CJD with PrP(Sc) type 2.

215 rovide insight into the etiology of sporadic CJD.

216 o those reported for human cases of sporadic CJD.

217 with probable AD, 26 with probable sporadic CJD, and 23 control patients for whom 14-3-3 protein, to

218 ease affected a younger cohort than sporadic CJD, and the early clinical course was dominated by psyc

219 rging illness in Washington or that sporadic CJD is more common in this state than in other regions o

220 There is little evidence that sporadic CJD is transmitted by blood transfusion.

221 ical evidence does not suggest that sporadic CJD is transmitted from person to person via blood trans

222 h a sub-optimal sensitivity for the sporadic CJD subtypes MM2C and MM2T, and a low sensitivity limite

223 to investigate the ability of these sporadic CJD molecular subtypes to propagate using brain-derived

224 e to vCJD and BSE prions but not to sporadic CJD prions.

225 CJD has a different pathogenesis to sporadic CJD.

226 ypic cultured GT1 cells, and unlike sporadic CJD isolates, kuru rapidly and stably infected these cel

227 (p=0.030) but not in patients with sporadic CJD (sCJD) or kuru.

228 brain regions from 8 patients with sporadic CJD (sCJD) was examined by histology, immunohistochemist

229 om a quarter of Swiss patients with sporadic CJD (sCJD).

230 for analysis, all 61 patients with sporadic CJD had positive RT-QuIC findings using OM or CSF sample

231 ts with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.

232 agnosis was likely, 2 patients with sporadic CJD tested positive (98.1% specificity; 95% CI, 93.3%-99

233 from 21 patients with vCJD, 27 with sporadic CJD, 42 with other neurological diseases, and 100 normal

234 F 12/13) in brains of subjects with sporadic CJD.

235 system cells similarly supported substantial CJD agent interference without PrPres.

236 nting with a biological ambiguity suggesting CJD, determination of CSF t-PrP levels increased diagnos

237 Positive cases in the suspected CJD group were further studied for antigen specificity u

238 ulation included 346 patients with suspected CJD and 49 patients with definite CJD.

239 relevant, number of patients with suspected CJD had potentially treatable disorders associated with

240 h vCJD and controls (patients with suspected CJD) were analysed.

241 In addition, SY-CJD prevented superinfection by sheep-derived Chandler (

242 tenuated Creutzfeldt-Jakob disease agent (SY-CJD) interferes with superinfection by a more virulent h

243 rse of the study, was found in 89% of tested CJD cases and 0% CJD mimics.

244 Tg(CJD) but not PrP KO mice were intrinsically more suscept

245 tic plasticity in hippocampal slices from Tg(CJD) mice, which model a genetic form of CJD.

246 of age, the magnitude of LTP increased in Tg(CJD) mice but decreased in PrP KO mice, indicating diver

247 A-dependent glutamatergic transmission in Tg(CJD) mice do not depend solely on PrP functional loss.

248 n the CA1 area of approximately 100-d-old Tg(CJD) mice was comparable to that of wild-type (WT) contr

249 beta-positive astrocytes increased in the Tg(CJD) hippocampus, and blocking IL-1 receptor signaling r

250 since the discovery a half century ago that CJD was transmissible.

251 profile of microglial changes induced by the CJD agent differed substantially from activation induced

252 dispersion, and possibly replication, of the CJD agent.

253 ternative diagnoses in more than half of the CJD mimic cases, and 10% had an immune-mediated encephal

254 velop a specific pattern of responses to the CJD agent, microglial markers may be exploited in the di

255 This CJD expression profile contrasted with that of uninfecte

256 ansmission of Abeta pathology in addition to CJD and suggests that healthy exposed individuals may al

257 against important differential diagnoses to CJD and may serve as a clinically useful diagnostic test

258 fluencing susceptibility of an individual to CJD.

259 e neuroimaging findings of VGKCC syndrome to CJD, recognizing VGKCC syndrome and the highly associate

260 e shorter incubation periods for variant (v) CJD prions, providing a more tractable model for studyin

261 Variant CJD prions showed prolonged incubation times between 300

262 reutzfeldt-Jakob disease (CJD) and 6 variant CJD brains.

263 in controls, sporadic CJD (sCJD) and variant CJD (vCJD) patients has identified three polymorphisms,

264 Creutzfeldt-Jakob disease (CJD) and variant CJD in humans.

265 Since clinical variant CJD is uniformly associated with tonsillar prion infecti

266 strain and are at risk of developing variant CJD.

267 Variant Creutzfeldt-Jakob disease (variant CJD) is difficult to distinguish from common psychiatric

268 ance of a novel human prion disease, variant CJD, and the clear experimental evidence that it is caus

269 ween species, suggest that the early variant CJD cases may have been exposed during the preclinical p

270 In 2002, 17 people died from variant CJD (vCJD) in the UK, compared with 20 in 2001 and 28 in

271 historically in kuru and recently in variant CJD.

272 data should inform attempts to model variant CJD epidemiology.

273 a, corticobasal degeneration and new variant CJD have been considered in affected individuals.

274 ia, including suspected cases of new variant CJD.

275 ghly consistent with, a diagnosis of variant CJD and negative in all patients subsequently confirmed

276 The arrival of variant CJD in the UK in the 1990s has intensified the search fo

277 hat the observation of four cases of variant CJD living in an area with a population of 1.5 million (

278 On second and third passages of variant CJD prions in Tg(MHu2M) mice, multiple strains of prions

279 We identified cases of variant CJD through the National CJD Surveillance Unit, and obta

280 It is unclear if a large epidemic of variant CJD will occur in the years ahead.

281 Up to Aug 31, 1998, 26 cases of variant CJD with onset in the UK (Northern Ireland not included)

282 disease (CJD); there was no case of variant CJD.

283 atures of this case overlap those of variant CJD.

284 Creutzfeldt-Jakob disease (sCJD), or variant CJD (vCJD) brains.

285 e spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecu

286 iopsy sample was positive for PrPSc, variant CJD could be diagnosed, which obviates the need for a br

287 nducted GWAS of sporadic CJD (sCJD), variant CJD (vCJD), iatrogenic CJD, inherited prion disease, kur

288 n disease emerged in the UK, termed "variant CJD", thought to be acquired by consumption of bovine sp

289 Our results also show that variant CJD has a different pathogenesis to sporadic CJD.

290 s do not support the hypotheses that variant CJD is an emerging illness in Washington or that sporadi

291 perimental exposure of astrocytes to variant CJD (vCJD), the kinetics of prion replication occur in a

292 2T, and a low sensitivity limited to variant CJD, Gerstmann-Straussler-Scheinker syndrome and fatal f

293 rotein in the urine of patients with variant CJD (vCJD) using protein misfolding by cyclic amplificat

294 ly similar) but can be infected with variant CJD prions, a human prion strain resulting from exposure

295 here is no evidence that people with variant CJD tended to live closer than the population as a whole

296 macaque experimentally infected with variant CJD.

297 viduals with CSF measures, including 93 with CJD, with 52 autopsy-verified samples (56%).

298 the ZBTB38-RASA2 locus were associated with CJD in the UK (rs295301, P = 3.13 x 10(-8); OR, 0.70) bu

299 ry CJD, accounting for >70% of families with CJD worldwide.

300 In patients with CJD, CSF t-PrP concentrations were decreased compared wi