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1 Creutzfeldt-Jakob disease (CJD) and autoimmune encephali
2 Creutzfeldt-Jakob disease (CJD) has been accidentally tr
3 Creutzfeldt-Jakob disease (CJD) in humans has been shown
4 Creutzfeldt-Jakob disease (CJD) is a neurodegenerative d
5 Creutzfeldt-Jakob disease (CJD) is a rare but invariably
6 Creutzfeldt-Jakob disease (CJD) is a rare progressive ne
7 Creutzfeldt-Jakob disease (CJD), the most common human p
8 nt diagnoses were Alzheimer's disease (18%), Creutzfeldt-Jakob disease (12%) and inflammatory disorde
9 rophic lateral sclerosis (FTD/ALS, n = 252), Creutzfeldt-Jakob disease (CJD, n = 239), Parkinson's di
11 rion protein disease with this feature after Creutzfeldt-Jakob disease, originally reported in 1920.
12 typical forms of Alzheimer disease (AD) and Creutzfeldt-Jakob disease (CJD) are clinically distingui
16 encephalopathy (BSE; "mad cow" disease) and Creutzfeldt-Jakob's disease, appears to be a beta-sheet-
17 cluding bovine spongiform encephalopathy and Creutzfeldt-Jakob disease, are usually characterized by
21 ey survive challenge with the human kuru and Creutzfeldt-Jakob agents as well as with scrapie agent i
24 including dementias such as Alzheimer's and Creutzfeldt-Jakob's disease and other central nervous sy
25 as diverse as Alzheimer's, Parkinson's, and Creutzfeldt-Jakob disease share a common pathogenetic me
27 s such as Alzheimer's (AD), Parkinson's, and Creutzfeldt-Jakob, and in animal diseases such as BSE.
28 nerative diseases, and arguably, scrapie and Creutzfeldt-Jakob disease prions represent the best ther
33 is related to various human diseases such as Creutzfeldt-Jakob disease and Gerstmann-Straussler-Schei
37 en implicated both in prion diseases such as Creutzfeldt-Jakob disease, where its monomeric cellular
39 maining patients, dementia with Lewy bodies, Creutzfeldt-Jakob disease, vascular or unclassified deme
40 replication of the infectious agents causing Creutzfeldt-Jakob disease (CJD), scrapie, and bovine spo
41 pletely resistant to both kuru and classical Creutzfeldt-Jakob disease (CJD) prions (which are closel
42 b disease (to date, 1,147 cases of confirmed Creutzfeldt-Jakob disease deaths in the United Kingdom s
43 More than two hundred individuals developed Creutzfeldt-Jakob disease (CJD) worldwide as a result of
45 m nasal brushings was accurate in diagnosing Creutzfeldt-Jakob disease and indicated substantial prio
46 between the invariably lethal prion disease Creutzfeldt-Jakob disease (CJD) and nonprion rapidly pro
47 g disease in deer and elk, and Kuru disease, Creutzfeldt-Jakob disease (CJD) and variant CJD in human
48 ation associated with one of these diseases [Creutzfeldt-Jakob disease (CJD)] that was exactly analog
52 ections with variant, sporadic, and familial Creutzfeldt-Jakob disease, in the presence of blood comp
53 sorders: fatal familial insomnia or familial Creutzfeldt-Jakob disease, depending upon the presence o
54 ile cerebrospinal fluid (CSF) biomarkers for Creutzfeldt-Jakob disease (CJD) are established and part
55 A test that detects the specific marker for Creutzfeldt-Jakob disease, the prion protein (PrP(CJD)),
56 s (PrPs) have shorter incubation periods for Creutzfeldt-Jakob disease (CJD) prions than mice express
57 PrP 129M/V polymorphism protects people from Creutzfeldt-Jakob disease, the Sup35p polymorphisms were
58 tes in the brains of patients suffering from Creutzfeldt-Jakob disease and related conditions, such a
59 ), frontotemporal lobar degeneration (FTLD), Creutzfeldt-Jakob disease (CJD), Alzheimer's disease (AD
60 s hypothalamic GT cells infected with the FU Creutzfeldt-Jakob disease agent, but not parallel mock c
62 ion diseases, including sheep scrapie, human Creutzfeldt-Jakob disease, and cervid chronic wasting di
66 dt-Jakob disease and in a case of iatrogenic Creutzfeldt-Jakob disease associated with growth hormone
68 , such as endotoxemia, sepsis, or iatrogenic Creutzfeldt-Jakob disease (to date, 1,147 cases of confi
70 ithin the cohort of patients with iatrogenic Creutzfeldt-Jakob disease in the UK suggests that there
72 pendent reports have claimed anticipation in Creutzfeldt-Jakob disease (CJD) caused by the c.598G > A
74 the clinicopathological diversity evident in Creutzfeldt-Jakob disease and whether different prion pr
76 uman brain extracts demonstrated that PrP in Creutzfeldt-Jakob disease (CJD) brains is cleaved by a c
77 Prion protein (PrP) plays a central role in Creutzfeldt-Jakob Disease (CJD) and other transmissible
79 le neurodegenerative conditions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spo
80 tal neurodegenerative disorders that include Creutzfeldt-Jakob disease in humans, scrapie in sheep an
81 al, neurodegenerative diseases which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spo
82 tions more common in the T/BG group included Creutzfeldt-Jakob disease, arbovirus, and Mycobacterium
83 eases of humans and other animals, including Creutzfeldt-Jakob disease and bovine spongiform encephal
84 fatal neurodegenerative conditions including Creutzfeldt-Jakob disease in humans and scrapie and bovi
85 ses numerous neurological diseases including Creutzfeldt-Jakob disease (CJD), but the aggregation mec
86 in many neurodegenerative diseases including Creutzfeldt-Jakob disease, motor neuron disease and Alzh
87 fatal neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in sheep an
88 ssible neurodegenerative disorders including Creutzfeldt-Jakob disease (CJD) in humans, is the conver
90 ldt-Jakob disease, and 2 of 2 with inherited Creutzfeldt-Jakob disease) but were negative in 43 of 43
93 urodegenerative disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinke
94 his case report from the Australian National Creutzfeldt-Jakob Disease Registry concerns a 61-year-ol
95 review of patients referred to the National Creutzfeldt-Jakob Disease Research & Surveillance Unit d
96 opagation of prions, the causative agents of Creutzfeldt-Jakob disease and other human prion diseases
98 on is believed responsible for most cases of Creutzfeldt-Jakob disease and for initiating the mad cow
99 tions can be detected early in the course of Creutzfeldt-Jakob Disease, and years before symptomatic
101 Early and accurate in vivo diagnosis of Creutzfeldt-Jakob disease (CJD) is necessary for quickly
103 umans have since developed a variant form of Creutzfeldt-Jakob disease (vCJD), also mostly in the Uni
104 f 14 patients with the E200K genetic form of Creutzfeldt-Jakob Disease, 20 healthy carriers of this m
105 sion and the appearance of a variant form of Creutzfeldt-Jakob disease, which has been linked to cons
107 is centrally involved in the pathogenesis of Creutzfeldt-Jakob Disease, and its anatomical connection
108 it was ineffective in slowing propagation of Creutzfeldt-Jakob disease prions in transgenic mice.
111 re immediate candidates for the treatment of Creutzfeldt-Jakob disease and other prion diseases.
112 ges that allowed early diagnosis of probable Creutzfeldt-Jakob disease before the characteristic clin
113 s (NDs), including Alzheimer's, Parkinson's, Creutzfeldt-Jakob, and Lou Gehrig's diseases, as well as
114 , diseases such as Alzheimer's, Parkinson's, Creutzfeldt-Jakob, and others display remarkable phenoty
118 rP(Sc) aggregates extracted from 60 sporadic Creutzfeldt-Jakob disease (CJD) and 6 variant CJD brains
119 iform encephalopathies (variant and sporadic Creutzfeldt-Jakob disease and genetic forms of prion dis
121 t disease phenotypes, identified as sporadic Creutzfeldt-Jakob disease (M/M2 sCJD) and sporadic fatal
124 ding, the most common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive
125 with the most common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive
126 al prion protein that characterizes sporadic Creutzfeldt-Jakob disease can be found in certain brain
128 und PrP species present in control, sporadic Creutzfeldt-Jakob disease (sCJD), or variant CJD (vCJD)
129 kob disease (15 of 15 with definite sporadic Creutzfeldt-Jakob disease, 13 of 14 with probable sporad
131 orts (FTLD, ALS, Alzheimer disease, sporadic Creutzfeldt-Jakob disease, Huntington disease-like syndr
133 an occur as an idiopathic disorder (sporadic Creutzfeldt-Jakob disease) or can be acquired, as is the
134 e most common human prion disorder, sporadic Creutzfeldt-Jakob disease (CJD), in which prions are for
135 cerebrospinal fluid (CSF) tests for sporadic Creutzfeldt-Jakob disease (sCJD) are based on the detect
136 and may be clinically mistaken for sporadic Creutzfeldt-Jakob disease because of a negative family h
139 n clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to h
142 ct subtypes have been identified in sporadic Creutzfeldt-Jakob disease (sCJD), based on the methionin
144 maging to study the white matter in sporadic Creutzfeldt-Jakob disease compared to healthy control su
146 fusivity within the white matter in sporadic Creutzfeldt-Jakob disease, suggesting possible primary i
150 59) and compare these with cases of sporadic Creutzfeldt-Jakob disease (n = 170) in the United Kingdo
151 tal insomnia (sFI) and a subtype of sporadic Creutzfeldt-Jakob disease (sCJD) identified as sCJDMM2,
153 QuIC differentiated 94% of cases of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 from the sCJD MM2 p
155 brains of two patients, who died of sporadic Creutzfeldt-Jakob disease (sCJD), contained either sCJD(
156 able at low levels in some cases of sporadic Creutzfeldt-Jakob disease and conversely, that the form
157 und in all of the major subtypes of sporadic Creutzfeldt-Jakob disease and in a case of iatrogenic Cr
163 long been used in the diagnosis of sporadic Creutzfeldt-Jakob disease; however, the characteristic w
164 re classified as either familial or sporadic Creutzfeldt-Jakob disease (CJD); there was no case of va
166 kob disease, 13 of 14 with probable sporadic Creutzfeldt-Jakob disease, and 2 of 2 with inherited Cre
168 Cumulatively, the data show that sporadic Creutzfeldt-Jakob disease PrP(Sc) is not a single confor
169 thought to confer susceptibility to sporadic Creutzfeldt-Jakob disease (rs1029273), all patients were
170 ns from the brains of patients with sporadic Creutzfeldt-Jakob disease (CJD) bind to very low-density
171 nd deer with those in subjects with sporadic Creutzfeldt-Jakob disease (CJD), as well as CJD-affected
173 the heart tissue of a patient with sporadic Creutzfeldt-Jakob Disease (sCJD), the most common form o
177 gregates derived from patients with sporadic Creutzfeldt-Jakob disease are taken up and degraded by i
178 , in brain samples from humans with sporadic Creutzfeldt-Jakob disease, as well as in rodents with ex
179 n the white matter of patients with sporadic Creutzfeldt-Jakob disease, mainly in the left hemisphere
180 patients with and patients without sporadic Creutzfeldt-Jakob disease and tested them using RT-QuIC,
183 Compared with a mouse model of transmissible Creutzfeldt-Jakob disease (CJD), the ataxia of Tg(A116V)
197 giform encephalopathy to people as a variant Creutzfeldt-Jakob disease (CJD), it becomes critical to
199 spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully m
205 rm encephalopathy (BSE) to humans as variant Creutzfeldt-Jakob disease (CJD) has affected over 100 in
206 spongiform encephalopathies, such as variant Creutzfeldt-Jakob disease, are believed to result from i
208 dy indicates a prototype blood-based variant Creutzfeldt-Jakob disease (vCJD) assay has sufficient se
209 m human post-mortem samples, of both variant Creutzfeldt-Jakob disease and Alzheimer's disease patien
210 vine spongiform encephalopathy (BSE)/variant Creutzfeldt-Jakob disease (vCJD), Nipah virus, several v
211 ease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having gu
212 he fatal neurodegenerative condition variant Creutzfeldt-Jakob disease (vCJD) and, based on recent hu
214 om donors who subsequently developed variant Creutzfeldt-Jakob disease and an asymptomatic red cell t
215 novel acquired human prion disease, variant Creutzfeldt-Jakob disease, is thought to result from ora
216 athy (BSE) and its human equivalent, variant Creutzfeldt-Jakob disease (vCJD), are caused by the same
218 re was no evidence of any death from variant Creutzfeldt-Jakob disease or from conditions that could
219 has been described in cases of human variant Creutzfeldt-Jakob disease (vCJD), experimental ovine bov
220 -fold dilution of 10% (wt/vol) human variant Creutzfeldt-Jakob disease brain homogenate, with >3,800-
221 s a single PrP(Sc) molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably r
222 Sc) that accumulates in the brain in variant Creutzfeldt-Jakob disease also contains a minority type
224 d with other transmission studies in variant Creutzfeldt-Jakob disease, including those on the spleen
228 35 deaths had been attributed to new variant Creutzfeldt-Jakob disease (nvCJD) in the United Kingdom,
232 ephalopathy to humans in the form of variant Creutzfeldt Jakob disease, have raised concerns about th
234 PrP(Sc) from autopsy-proved cases of variant Creutzfeldt-Jakob disease (n = 59) and compare these wit
235 truments from, a deceased carrier of variant Creutzfeldt-Jakob disease (vCJD) can be followed up to q
237 Person-to-person transmission of variant Creutzfeldt-Jakob disease (vCJD) has occurred through bl
238 relevant to the current outbreak of variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom,
240 cal variation in the distribution of variant Creutzfeldt-Jakob disease (vCJD) might indicate the tran
243 reasing concern since the reports of variant Creutzfeldt-Jakob disease (vCJD) transmission through bl
244 fication of possible transmission of variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion h
248 cuses on transfusion-transmission of variant Creutzfeldt-Jakob disease by red cell preparations.
249 by this review, the transmission of variant Creutzfeldt-Jakob disease by transfusion has been confir
250 a precaution against transmission of variant Creutzfeldt-Jakob disease by transfusion of domestic blo
253 vious definite and probable cases of variant Creutzfeldt-Jakob disease have been methionine homozygot
255 Risk assessments for transmission of variant Creutzfeldt-Jakob disease predicted that leukocyte reduc
256 PrP(Sc) was found in all 21 cases of variant Creutzfeldt-Jakob disease tested, irrespective of brain
259 ular system is a defining feature of variant Creutzfeldt-Jakob disease, but that the biochemical isof
260 fusion recipient, who did not die of variant Creutzfeldt-Jakob disease, has been identified with prio
264 ee cases of probable transmission of variant Creutzfeldt-Jakob infectivity by transfusion of red cell
265 lity to, and clinical expression of, variant Creutzfeldt-Jakob disease (vCJD) is essential for future
266 either Creutzfeldt-Jakob disease or variant Creutzfeldt-Jakob disease are transmitted by transfusion
268 mples, from prion-affected patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD
269 We report a case of preclinical variant Creutzfeldt-Jakob disease (vCJD) in a patient who died f
270 and findings were followed-up in six variant Creutzfeldt-Jakob disease cases with 9.4 T high-resoluti
271 r significant numbers of subclinical variant Creutzfeldt-Jakob disease individuals in at least the Un
275 enotype individual can propagate the variant Creutzfeldt-Jakob disease agent and that the infectious
277 Back-calculation analysis of the variant Creutzfeldt-Jakob disease epidemic in the United Kingdom
279 cell preparations are applicable to variant Creutzfeldt-Jakob in humans then a method for rendering
280 timates for the risk of transmitting variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion h
281 en PrP(Sc) types was maintained when variant Creutzfeldt-Jakob disease was transmitted to wild-type m
282 of humans and animals, one of which (variant Creutzfeldt-Jakob disease) is known to be a zoonotic for
283 sceptible to prions from humans with variant Creutzfeldt-Jakob disease (CJD); on second passage in Tg
286 in lymphoid tissues of patients with variant Creutzfeldt-Jakob disease (vCJD), sheep with natural scr
288 e only in the urine of patients with variant Creutzfeldt-Jakob disease and had the typical electropho
289 samples obtained from patients with variant Creutzfeldt-Jakob disease and in none of the 224 urine s
290 samples obtained from patients with variant Creutzfeldt-Jakob disease contained minute quantities of
292 phic codon in humans associated with variant Creutzfeldt-Jakob disease, pulls the N terminus into the
293 tected in the urine of patients with variant Creutzfeldt-Jakob disease, we used the protein misfoldin
294 rent understanding of the risks of (variant) Creutzfeldt-Jakob disease transmission via dental practi
295 First, we successfully propagated various Creutzfeldt-Jakob disease (CJD) isolates (sporadic, vari
296 myoclonic jerks develop in individuals with Creutzfeldt-Jakob disease (CJD), an incurable brain diso
297 f growth hormone derived from a patient with Creutzfeldt-Jakob disease expressing prion protein valin
298 ings were positive in 30 of 31 patients with Creutzfeldt-Jakob disease (15 of 15 with definite sporad
300 PrP(TSE)) when exposed to medium spiked with Creutzfeldt-Jakob disease brain homogenate, resulting in
301 t were negative in 43 of 43 patients without Creutzfeldt-Jakob disease, indicating a sensitivity of 9
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