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2 actors in response to the foreign Creutzfeld-Jakob disease agent, it is likely that innate immunity u
3 on of the attenuated SY strain of Creutzfeld-Jakob disease in mice could delay clinical signs and wid
6 to humans in the form of variant Creutzfeldt Jakob disease, have raised concerns about the zoonotic p
15 al sclerosis (FTD/ALS, n = 252), Creutzfeldt-Jakob disease (CJD, n = 239), Parkinson's disease (PD, n
18 hallenge with the human kuru and Creutzfeldt-Jakob agents as well as with scrapie agent isolated from
19 ms of Alzheimer disease (AD) and Creutzfeldt-Jakob disease (CJD) are clinically distinguishable, atyp
22 eases, and arguably, scrapie and Creutzfeldt-Jakob disease prions represent the best therapeutic targ
23 as Alzheimer's, Parkinson's, and Creutzfeldt-Jakob disease share a common pathogenetic mechanism invo
24 ne spongiform encephalopathy and Creutzfeldt-Jakob disease, are usually characterized by the accumula
29 itions including Alzheimer's and Creutzfeldt-Jakob diseases represent, on this basis, pathological ca
32 ementias such as Alzheimer's and Creutzfeldt-Jakob's disease and other central nervous system disease
33 thy (BSE; "mad cow" disease) and Creutzfeldt-Jakob's disease, appears to be a beta-sheet-rich amyloid
39 o various human diseases such as Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker syndrom
42 Human prion diseases such as Creutzfeldt-Jakob disease are transmissible brain proteinopathies, c
43 d both in prion diseases such as Creutzfeldt-Jakob disease, where its monomeric cellular isoform (PrP
44 ction of mice with an attenuated Creutzfeldt-Jakob disease agent (SY-CJD) interferes with superinfect
46 of the infectious agents causing Creutzfeldt-Jakob disease (CJD), scrapie, and bovine spongiform ence
47 stant to both kuru and classical Creutzfeldt-Jakob disease (CJD) prions (which are closely similar) b
48 o date, 1,147 cases of confirmed Creutzfeldt-Jakob disease deaths in the United Kingdom since 1990 ha
49 wo hundred individuals developed Creutzfeldt-Jakob disease (CJD) worldwide as a result of treatment,
51 hings was accurate in diagnosing Creutzfeldt-Jakob disease and indicated substantial prion seeding ac
52 invariably lethal prion disease Creutzfeldt-Jakob disease (CJD) and nonprion rapidly progressive dem
54 ated with one of these diseases [Creutzfeldt-Jakob disease (CJD)] that was exactly analogous to a pre
57 al familial insomnia or familial Creutzfeldt-Jakob disease, depending upon the presence of Met or Val
59 pinal fluid (CSF) biomarkers for Creutzfeldt-Jakob disease (CJD) are established and partly included
60 e shorter incubation periods for Creutzfeldt-Jakob disease (CJD) prions than mice expressing full-len
61 detects the specific marker for Creutzfeldt-Jakob disease, the prion protein (PrP(CJD)), by means of
62 rains of patients suffering from Creutzfeldt-Jakob disease and related conditions, such as Gerstmann-
63 olymorphism protects people from Creutzfeldt-Jakob disease, the Sup35p polymorphisms were selected to
64 poral lobar degeneration (FTLD), Creutzfeldt-Jakob disease (CJD), Alzheimer's disease (AD), Parkinson
65 ic GT cells infected with the FU Creutzfeldt-Jakob disease agent, but not parallel mock controls, dis
69 dotoxemia, sepsis, or iatrogenic Creutzfeldt-Jakob disease (to date, 1,147 cases of confirmed Creutzf
70 ease and in a case of iatrogenic Creutzfeldt-Jakob disease associated with growth hormone therapy.
73 hort of patients with iatrogenic Creutzfeldt-Jakob disease in the UK suggests that there was a point
74 incubation period of iatrogenic Creutzfeldt-Jakob disease is significantly different between all thr
77 in (PrP) plays a central role in Creutzfeldt-Jakob Disease (CJD) and other transmissible spongiform e
78 xtracts demonstrated that PrP in Creutzfeldt-Jakob disease (CJD) brains is cleaved by a cellular prot
79 rts have claimed anticipation in Creutzfeldt-Jakob disease (CJD) caused by the c.598G > A mutation in
82 athological diversity evident in Creutzfeldt-Jakob disease and whether different prion protein types
84 enerative diseases which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform ence
85 nerative conditions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform ence
86 enerative disorders that include Creutzfeldt-Jakob disease in humans, scrapie in sheep and goats, and
87 ommon in the T/BG group included Creutzfeldt-Jakob disease, arbovirus, and Mycobacterium tuberculosis
88 degenerative disorders including Creutzfeldt-Jakob disease (CJD) in humans, is the conversion of the
89 neurological diseases including Creutzfeldt-Jakob disease (CJD), but the aggregation mechanisms rema
92 egenerative conditions including Creutzfeldt-Jakob disease in humans and scrapie and bovine spongifor
93 degenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in sheep and bovine spo
94 odegenerative diseases including Creutzfeldt-Jakob disease, motor neuron disease and Alzheimer's dise
95 sease, and 2 of 2 with inherited Creutzfeldt-Jakob disease) but were negative in 43 of 43 patients wi
97 ive disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome (
98 ort from the Australian National Creutzfeldt-Jakob Disease Registry concerns a 61-year-old British-bo
99 atients referred to the National Creutzfeldt-Jakob Disease Research & Surveillance Unit during the pe
101 lthough surgical transmission of Creutzfeldt-Jakob disease (CJD) has been demonstrated, these iatroge
102 nd accurate in vivo diagnosis of Creutzfeldt-Jakob disease (CJD) is necessary for quickly distinguish
104 ince developed a variant form of Creutzfeldt-Jakob disease (vCJD), also mostly in the United Kingdom,
105 prions, the causative agents of Creutzfeldt-Jakob disease and other human prion diseases, requires p
108 s with the E200K genetic form of Creutzfeldt-Jakob Disease, 20 healthy carriers of this mutation that
109 involved in the pathogenesis of Creutzfeldt-Jakob Disease, and its anatomical connections are suffic
110 detected early in the course of Creutzfeldt-Jakob Disease, and years before symptomatic onset in mut
111 appearance of a variant form of Creutzfeldt-Jakob disease, which has been linked to consumption of p
114 owed early diagnosis of probable Creutzfeldt-Jakob disease before the characteristic clinical picture
115 luding Alzheimer's, Parkinson's, Creutzfeldt-Jakob, and Lou Gehrig's diseases, as well as the tauopat
116 uch as Alzheimer's, Parkinson's, Creutzfeldt-Jakob, and others display remarkable phenotypic heteroge
118 brains of patients with sporadic Creutzfeldt-Jakob disease (CJD) bind to very low-density (VLDL) and
121 those in subjects with sporadic Creutzfeldt-Jakob disease (CJD), as well as CJD-affected subjects wh
122 n human prion disorder, sporadic Creutzfeldt-Jakob disease (CJD), in which prions are formed spontane
124 d as either familial or sporadic Creutzfeldt-Jakob disease (CJD); there was no case of variant CJD.
125 enotypes, identified as sporadic Creutzfeldt-Jakob disease (M/M2 sCJD) and sporadic fatal insomnia (s
126 are these with cases of sporadic Creutzfeldt-Jakob disease (n = 170) in the United Kingdom over the p
127 onfer susceptibility to sporadic Creutzfeldt-Jakob disease (rs1029273), all patients were homozygous
128 were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alter
129 l fluid (CSF) tests for sporadic Creutzfeldt-Jakob disease (sCJD) are based on the detection of surro
130 (sFI) and a subtype of sporadic Creutzfeldt-Jakob disease (sCJD) identified as sCJDMM2, which share
132 ntiated 94% of cases of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 from the sCJD MM2 phenotype, an
135 st common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia.
136 st common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia.
137 have been identified in sporadic Creutzfeldt-Jakob disease (sCJD), based on the methionine/valine pol
138 o patients, who died of sporadic Creutzfeldt-Jakob disease (sCJD), contained either sCJD(MM1) or sCJD
140 ies present in control, sporadic Creutzfeldt-Jakob disease (sCJD), or variant CJD (vCJD) brains.
141 issue of a patient with sporadic Creutzfeldt-Jakob Disease (sCJD), the most common form of human prio
146 levels in some cases of sporadic Creutzfeldt-Jakob disease and conversely, that the form of abnormal
147 alopathies (variant and sporadic Creutzfeldt-Jakob disease and genetic forms of prion disease), patie
148 f the major subtypes of sporadic Creutzfeldt-Jakob disease and in a case of iatrogenic Creutzfeldt-Ja
149 wenty-six patients with sporadic Creutzfeldt-Jakob disease and nine age- and gender-matched healthy c
150 th and patients without sporadic Creutzfeldt-Jakob disease and tested them using RT-QuIC, an ultrasen
151 ived from patients with sporadic Creutzfeldt-Jakob disease are taken up and degraded by immortalized
152 clinically mistaken for sporadic Creutzfeldt-Jakob disease because of a negative family history.
153 tein that characterizes sporadic Creutzfeldt-Jakob disease can be found in certain brain regions of c
154 udy the white matter in sporadic Creutzfeldt-Jakob disease compared to healthy control subjects and t
155 Definite diagnosis of sporadic Creutzfeldt-Jakob disease in living patients remains a challenge.
159 ely, the data show that sporadic Creutzfeldt-Jakob disease PrP(Sc) is not a single conformational ent
160 Cases diagnosed as sporadic Creutzfeldt-Jakob disease with atypical neuropathology were also rev
161 an idiopathic disorder (sporadic Creutzfeldt-Jakob disease) or can be acquired, as is the case for va
162 (15 of 15 with definite sporadic Creutzfeldt-Jakob disease, 13 of 14 with probable sporadic Creutzfel
163 13 of 14 with probable sporadic Creutzfeldt-Jakob disease, and 2 of 2 with inherited Creutzfeldt-Jak
164 amples from humans with sporadic Creutzfeldt-Jakob disease, as well as in rodents with experimental p
166 ALS, Alzheimer disease, sporadic Creutzfeldt-Jakob disease, Huntington disease-like syndrome, and oth
167 matter of patients with sporadic Creutzfeldt-Jakob disease, mainly in the left hemisphere, with a str
168 hin the white matter in sporadic Creutzfeldt-Jakob disease, suggesting possible primary involvement o
181 sed in the diagnosis of sporadic Creutzfeldt-Jakob disease; however, the characteristic waveforms do
185 h a mouse model of transmissible Creutzfeldt-Jakob disease (CJD), the ataxia of Tg(A116V) mice is mor
186 pathy (BSE) to humans as variant Creutzfeldt-Jakob disease (CJD) has affected over 100 individuals, a
188 halopathy to people as a variant Creutzfeldt-Jakob disease (CJD), it becomes critical to identify cel
190 prions from humans with variant Creutzfeldt-Jakob disease (CJD); on second passage in Tg(BoPrP) mice
191 autopsy-proved cases of variant Creutzfeldt-Jakob disease (n = 59) and compare these with cases of s
192 rP(Sc) molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably resulting fro
194 rodegenerative condition variant Creutzfeldt-Jakob disease (vCJD) and, based on recent human prevalen
195 a prototype blood-based variant Creutzfeldt-Jakob disease (vCJD) assay has sufficient sensitivity an
196 m, a deceased carrier of variant Creutzfeldt-Jakob disease (vCJD) can be followed up to quantify tran
201 o-person transmission of variant Creutzfeldt-Jakob disease (vCJD) has occurred through blood transfus
203 number of patients with variant Creutzfeldt-Jakob disease (vCJD) have been treated with intraventicu
204 rt a case of preclinical variant Creutzfeldt-Jakob disease (vCJD) in a patient who died from a non-ne
205 to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protect
206 the current outbreak of variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom, because all
213 clinical expression of, variant Creutzfeldt-Jakob disease (vCJD) is essential for future management
214 ns have been raised that variant Creutzfeldt-Jakob disease (vCJD) might be transmissible by blood tra
215 n in the distribution of variant Creutzfeldt-Jakob disease (vCJD) might indicate the transmission rou
216 prion-affected patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD (iCJD) n = 1
217 encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully maintained up
218 The transmission of variant Creutzfeldt-Jakob disease (vCJD) through blood transfusions has crea
221 ern since the reports of variant Creutzfeldt-Jakob disease (vCJD) transmission through blood transfus
222 possible transmission of variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion has caused co
223 the risk of transmitting variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion have relied l
228 nd its human equivalent, variant Creutzfeldt-Jakob disease (vCJD), are caused by the same strain of i
229 cribed in cases of human variant Creutzfeldt-Jakob disease (vCJD), experimental ovine bovine spongifo
230 orm encephalopathy (BSE)/variant Creutzfeldt-Jakob disease (vCJD), Nipah virus, several viral hemorrh
231 tissues of patients with variant Creutzfeldt-Jakob disease (vCJD), sheep with natural scrapie or rode
235 vidual can propagate the variant Creutzfeldt-Jakob disease agent and that the infectious agent can be
237 umulates in the brain in variant Creutzfeldt-Jakob disease also contains a minority type 1 component.
238 -mortem samples, of both variant Creutzfeldt-Jakob disease and Alzheimer's disease patients, also sug
240 o subsequently developed variant Creutzfeldt-Jakob disease and an asymptomatic red cell transfusion r
241 e urine of patients with variant Creutzfeldt-Jakob disease and had the typical electrophoretic profil
242 ained from patients with variant Creutzfeldt-Jakob disease and in none of the 224 urine samples obtai
243 on of 10% (wt/vol) human variant Creutzfeldt-Jakob disease brain homogenate, with >3,800-fold binding
246 against transmission of variant Creutzfeldt-Jakob disease by transfusion of domestic blood or red bl
247 Recent evidence that variant Creutzfeldt-Jakob disease can be transmitted by transfusion of red c
248 were followed-up in six variant Creutzfeldt-Jakob disease cases with 9.4 T high-resolution magnetiza
250 ained from patients with variant Creutzfeldt-Jakob disease contained minute quantities of PrP(Sc).
251 culation analysis of the variant Creutzfeldt-Jakob disease epidemic in the United Kingdom is used to
252 The recent emergence of variant Creutzfeldt-Jakob disease has led to major public health concerns, a
253 The outbreak of new variant Creutzfeldt-Jakob disease has raised the specter of a potentially la
255 te and probable cases of variant Creutzfeldt-Jakob disease have been methionine homozygotes (MM).
258 t numbers of subclinical variant Creutzfeldt-Jakob disease individuals in at least the United Kingdom
259 idence of any death from variant Creutzfeldt-Jakob disease or from conditions that could be confused
260 ents for transmission of variant Creutzfeldt-Jakob disease predicted that leukocyte reduction would b
261 Evidence suggests that variant Creutzfeldt-Jakob disease prions circulate in body fluids from peopl
262 found in all 21 cases of variant Creutzfeldt-Jakob disease tested, irrespective of brain region exami
266 ypes was maintained when variant Creutzfeldt-Jakob disease was transmitted to wild-type mice and was
267 We report a case of variant Creutzfeldt-Jakob disease(vCJD) in a 74-year old man in whom diagnos
268 d animals, one of which (variant Creutzfeldt-Jakob disease) is known to be a zoonotic form of the cat
269 ncephalopathies, such as variant Creutzfeldt-Jakob disease, are believed to result from infectious fo
270 is a defining feature of variant Creutzfeldt-Jakob disease, but that the biochemical isoform of PrP(S
271 an effort to prevent new variant Creutzfeldt-Jakob disease, certain "specified offals," including neu
272 ient, who did not die of variant Creutzfeldt-Jakob disease, has been identified with prion protein de
273 transmission studies in variant Creutzfeldt-Jakob disease, including those on the spleen and brain o
274 red human prion disease, variant Creutzfeldt-Jakob disease, is thought to result from oral exposure t
276 n humans associated with variant Creutzfeldt-Jakob disease, pulls the N terminus into the sheet.
277 e urine of patients with variant Creutzfeldt-Jakob disease, we used the protein misfolding cyclic amp
278 f mid-2016, 231 cases of variant Creutzfeldt-Jakob disease-the human form of a prion disease of cattl
289 ations are applicable to variant Creutzfeldt-Jakob in humans then a method for rendering human red ce
290 probable transmission of variant Creutzfeldt-Jakob infectivity by transfusion of red cell preparation
291 anding of the risks of (variant) Creutzfeldt-Jakob disease transmission via dental practice, and whet
292 successfully propagated various Creutzfeldt-Jakob disease (CJD) isolates (sporadic, variant, and iat
293 sitive in 30 of 31 patients with Creutzfeldt-Jakob disease (15 of 15 with definite sporadic Creutzfel
294 erks develop in individuals with Creutzfeldt-Jakob disease (CJD), an incurable brain disorder caused
295 en exposed to medium spiked with Creutzfeldt-Jakob disease brain homogenate, resulting in a coarse gr
298 ive in 43 of 43 patients without Creutzfeldt-Jakob disease, indicating a sensitivity of 97% (95% conf
299 traussler syndrome and hereditary Creuzfeldt-Jakob disease, tau mutations cause autosomal dominant fr
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