ライフサイエンスコーパス: CJD

1 CJD-infected microglia also displayed morphological chan

2 CJD-MM1/MV1 cases displayed higher neurogranin levels th

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

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

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

6 10-24 brain regions (100%) examined from 28 CJD patients.

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

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

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

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

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

12 gree of neuronal damage in brain tissue in a CJD subtype manner.

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

14 and from those seen in sporadic and acquired CJD.

15 Prion RT-QuIC was positive in all CJD PNS samples, whereas western blotting detected PrP(S

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

17 ological controls (NCs, n=64), AD (n=46) and CJD (n=81).

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

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

20 atypical AD phenotypes wrongly classified as CJD.

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

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

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

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

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

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

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

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

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

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

31 suspected CJD and 49 patients with definite CJD.

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

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

34 be considered and excluded before diagnosing CJD.

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

36 .62 to 0.82), and were able to differentiate CJD from AD (p<0.001, AUC 0.85, 95% CI 0.78 to 0.92).

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

76 s disease, 6 with Creutzfeldt-Jakob disease (CJD)).

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

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

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

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

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

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

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

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

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

86 cular subtypes of Creutzfeldt-Jakob disease (CJD), we applied 3 different anti-PrP antibodies.

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

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

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

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

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

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

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

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

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

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

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

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

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

100 Neurogranin was increased at early CJD disease stages and was a good prognostic marker of s

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

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

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

104 ssues of patients with sporadic and familial CJD.

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

106 ional octapeptide repeats linked to familial CJD.

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

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

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

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

111 omparison to all other categories except for CJD.

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

113 ble disorders are most commonly mistaken for CJD.

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

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

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

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

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

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

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

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

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

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

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

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

126 1 VV-2 type sporadic CJD and 7 of 30 genetic CJD cases showed vagal PrP(Sc) immunodeposits with disti

127 e vagus nerve in 162 sporadic and 30 genetic CJD cases.

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

129 derived from human iPSCs can replicate human CJD prions.

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

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

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

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

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

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

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

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

138 In CJD, neurogranin positively correlated with tau (r=0.55,

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

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

141 neurogranin concentrations were increased in CJD (4.75 times of NC; p<0.001, area under curve (AUC),

142 CSF tau was increased in CJD (41 times of NC) and in AD (3.1 times of NC), both a

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

144 13 kDa midspan PrP fragment, not observed in CJD.

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

146 Thus, PrP(Sc) in CJD affects the vagus nerve analogously to alpha-synucle

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

148 uced levels in AD, and more significantly in CJD, where they correlated with synaptic and axonal mark

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

150 a good prognostic marker of survival time in CJD.

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

152 able or definite sCJD identified at national CJD referral centres) with a two-stage study design usin

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

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

155 The National CJD Surveillance Unit prospectively identified 84 people

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

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

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

159 ), 28 control brains of individuals with non-CJD neurodegenerative diseases and 10 normal brains.

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

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

162 tive and gestational tissues in this case of CJD.

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

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

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

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

167 regarded as sufficient for the diagnosis of CJD.

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

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

170 reen brains of cornea donors for evidence of CJD.

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

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

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

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

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

176 re underlie susceptibility to these forms of CJD.

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

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

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

180 replication of prions from brain samples of CJD patients.

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

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

183 rapid neurologic deterioration suggestive of CJD.

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

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

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

187 s who fulfill clinical criteria for possible CJD.

188 diagnostic criteria for probable or possible CJD.

189 diagnostic criteria of probable or possible CJD.

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

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

192 appeared allowed for a diagnosis of probable CJD.

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

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

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

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

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

198 22372, which was susceptible to sporadic (s) CJD prions in approximately 110 days.

199 Sporadic CJD and BSE agents and representative scrapie agents wer

200 = 20, iatrogenic CJD (iCJD) n = 11, sporadic CJD (sCJD) n = 23, familial CJD (gCJD) n = 17, fatal fam

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

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

203 eposition of PrP(Sc) in genetic and sporadic CJD argues against uniform mechanisms of propagation of

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

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

206 or additional factors may determine sporadic CJD subtype.

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

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

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

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

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

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

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

214 fication of disease subtype also in sporadic CJD.

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

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

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

218 A molecular classification of sporadic CJD is proposed.

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

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

221 ion disease across the diversity of sporadic CJD subtypes.

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

223 rovide insight into the etiology of sporadic CJD.

224 o those reported for human cases of sporadic CJD.

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

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

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

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

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

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

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

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

233 Four of 31 VV-2 type sporadic CJD and 7 of 30 genetic CJD cases showed vagal PrP(Sc) i

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

263 This CJD expression profile contrasted with that of uninfecte

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

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

266 fluencing susceptibility of an individual to CJD.

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

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

269 Variant CJD prions showed prolonged incubation times between 300

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

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

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

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

274 strain and are at risk of developing variant CJD.

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

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

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

278 historically in kuru and recently in variant CJD.

279 data should inform attempts to model variant CJD epidemiology.

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

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

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

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

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

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

286 atures of this case overlap those of variant CJD.

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

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

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

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

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

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

293 2T, and a low sensitivity limited to variant CJD, Gerstmann-Straussler-Scheinker syndrome and fatal f

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

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

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

297 macaque experimentally infected with variant CJD.

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

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

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