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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 esthesia, pure dementia GSS, and Creutzfeldt-Jakob disease-like GSS); GSS may be more common than pre
30 ementias such as Alzheimer's and Creutzfeldt-Jakob's disease and other central nervous system disease
31 thy (BSE; "mad cow" disease) and Creutzfeldt-Jakob's disease, appears to be a beta-sheet-rich amyloid
37 o various human diseases such as Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker syndrom
40 Human prion diseases such as Creutzfeldt-Jakob disease are transmissible brain proteinopathies, c
41 d both in prion diseases such as Creutzfeldt-Jakob disease, where its monomeric cellular isoform (PrP
42 ction of mice with an attenuated Creutzfeldt-Jakob disease agent (SY-CJD) interferes with superinfect
44 of the infectious agents causing Creutzfeldt-Jakob disease (CJD), scrapie, and bovine spongiform ence
45 stant to both kuru and classical Creutzfeldt-Jakob disease (CJD) prions (which are closely similar) b
46 o date, 1,147 cases of confirmed Creutzfeldt-Jakob disease deaths in the United Kingdom since 1990 ha
47 wo hundred individuals developed Creutzfeldt-Jakob disease (CJD) worldwide as a result of treatment,
49 hings was accurate in diagnosing Creutzfeldt-Jakob disease and indicated substantial prion seeding ac
50 invariably lethal prion disease Creutzfeldt-Jakob disease (CJD) and nonprion rapidly progressive dem
51 ated with one of these diseases [Creutzfeldt-Jakob disease (CJD)] that was exactly analogous to a pre
54 al familial insomnia or familial Creutzfeldt-Jakob disease, depending upon the presence of Met or Val
56 pinal fluid (CSF) biomarkers for Creutzfeldt-Jakob disease (CJD) are established and partly included
57 e shorter incubation periods for Creutzfeldt-Jakob disease (CJD) prions than mice expressing full-len
58 detects the specific marker for Creutzfeldt-Jakob disease, the prion protein (PrP(CJD)), by means of
59 rains of patients suffering from Creutzfeldt-Jakob disease and related conditions, such as Gerstmann-
60 cal syndrome is so distinct from Creutzfeldt-Jakob disease, and biomarkers of the early stages of dis
61 olymorphism protects people from Creutzfeldt-Jakob disease, the Sup35p polymorphisms were selected to
62 poral lobar degeneration (FTLD), Creutzfeldt-Jakob disease (CJD), Alzheimer's disease (AD), Parkinson
63 ic GT cells infected with the FU Creutzfeldt-Jakob disease agent, but not parallel mock controls, dis
67 dotoxemia, sepsis, or iatrogenic Creutzfeldt-Jakob disease (to date, 1,147 cases of confirmed Creutzf
68 e French epidemics of iatrogenic Creutzfeldt-Jakob disease after growth hormone (GH) treatment provid
69 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
76 both with and without iatrogenic Creutzfeldt-Jakob disease; however, there is little information rega
77 poradic, variant, and iatrogenic Creutzfeldt-Jakob disease; kuru; inherited prion disease; sheep scra
79 in (PrP) plays a central role in Creutzfeldt-Jakob Disease (CJD) and other transmissible spongiform e
80 xtracts demonstrated that PrP in Creutzfeldt-Jakob disease (CJD) brains is cleaved by a cellular prot
81 rts have claimed anticipation in Creutzfeldt-Jakob disease (CJD) caused by the c.598G > A mutation in
84 athological diversity evident in Creutzfeldt-Jakob disease and whether different prion protein types
86 enerative diseases which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform ence
87 enerative disorders that include Creutzfeldt-Jakob disease in humans, scrapie in sheep and goats, and
88 ommon in the T/BG group included Creutzfeldt-Jakob disease, arbovirus, and Mycobacterium tuberculosis
89 degenerative disorders including Creutzfeldt-Jakob disease (CJD) in humans, is the conversion of the
90 neurological diseases including Creutzfeldt-Jakob disease (CJD), but the aggregation mechanisms rema
93 egenerative conditions including Creutzfeldt-Jakob disease in humans and scrapie and bovine spongifor
94 degenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in sheep and bovine spo
95 odegenerative diseases including Creutzfeldt-Jakob disease, motor neuron disease and Alzheimer's dise
96 sease, and 2 of 2 with inherited Creutzfeldt-Jakob disease) but were negative in 43 of 43 patients wi
97 mans), responsible for inherited Creutzfeldt-Jakob disease, finds that the mutation lowers metal ion
99 ive disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome (
100 ort from the Australian National Creutzfeldt-Jakob Disease Registry concerns a 61-year-old British-bo
101 atients referred to the National Creutzfeldt-Jakob Disease Research & Surveillance Unit during the pe
103 lthough surgical transmission of Creutzfeldt-Jakob disease (CJD) has been demonstrated, these iatroge
104 nd accurate in vivo diagnosis of Creutzfeldt-Jakob disease (CJD) is necessary for quickly distinguish
105 forms and molecular subtypes of Creutzfeldt-Jakob disease (CJD), we applied 3 different anti-PrP ant
107 ince developed a variant form of Creutzfeldt-Jakob disease (vCJD), also mostly in the United Kingdom,
108 prions, the causative agents of Creutzfeldt-Jakob disease and other human prion diseases, requires p
110 s with the E200K genetic form of Creutzfeldt-Jakob Disease, 20 healthy carriers of this mutation that
111 involved in the pathogenesis of Creutzfeldt-Jakob Disease, and its anatomical connections are suffic
112 detected early in the course of Creutzfeldt-Jakob Disease, and years before symptomatic onset in mut
113 appearance of a variant form of Creutzfeldt-Jakob disease, which has been linked to consumption of p
116 owed early diagnosis of probable Creutzfeldt-Jakob disease before the characteristic clinical picture
117 luding Alzheimer's, Parkinson's, Creutzfeldt-Jakob, and Lou Gehrig's diseases, as well as the tauopat
118 uch as Alzheimer's, Parkinson's, Creutzfeldt-Jakob, and others display remarkable phenotypic heteroge
120 brains of patients with sporadic Creutzfeldt-Jakob disease (CJD) bind to very low-density (VLDL) and
123 those in subjects with sporadic Creutzfeldt-Jakob disease (CJD), as well as CJD-affected subjects wh
124 n human prion disorder, sporadic Creutzfeldt-Jakob disease (CJD), in which prions are formed spontane
126 d as either familial or sporadic Creutzfeldt-Jakob disease (CJD); there was no case of variant CJD.
127 are these with cases of sporadic Creutzfeldt-Jakob disease (n = 170) in the United Kingdom over the p
128 onfer susceptibility to sporadic Creutzfeldt-Jakob disease (rs1029273), all patients were homozygous
129 were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alter
130 l fluid (CSF) tests for sporadic Creutzfeldt-Jakob disease (sCJD) are based on the detection of surro
132 (sFI) and a subtype of sporadic Creutzfeldt-Jakob disease (sCJD) identified as sCJDMM2, which share
133 an prion disease cases, sporadic Creutzfeldt-Jakob disease (sCJD) is the prevalent human prion diseas
134 ntiated 94% of cases of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 from the sCJD MM2 phenotype, an
138 ed study of a cohort of sporadic Creutzfeldt-Jakob disease (sCJD) VV1-2 type-mixed cases (valine homo
139 st common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia.
140 st common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia.
141 have been identified in sporadic Creutzfeldt-Jakob disease (sCJD), based on the methionine/valine pol
142 o patients, who died of sporadic Creutzfeldt-Jakob disease (sCJD), contained either sCJD(MM1) or sCJD
144 ies present in control, sporadic Creutzfeldt-Jakob disease (sCJD), or variant CJD (vCJD) brains.
145 issue of a patient with sporadic Creutzfeldt-Jakob Disease (sCJD), the most common form of human prio
151 levels in some cases of sporadic Creutzfeldt-Jakob disease and conversely, that the form of abnormal
152 alopathies (variant and sporadic Creutzfeldt-Jakob disease and genetic forms of prion disease), patie
153 f the major subtypes of sporadic Creutzfeldt-Jakob disease and in a case of iatrogenic Creutzfeldt-Ja
154 wenty-six patients with sporadic Creutzfeldt-Jakob disease and nine age- and gender-matched healthy c
155 th and patients without sporadic Creutzfeldt-Jakob disease and tested them using RT-QuIC, an ultrasen
156 ived from patients with sporadic Creutzfeldt-Jakob disease are taken up and degraded by immortalized
157 clinically mistaken for sporadic Creutzfeldt-Jakob disease because of a negative family history.
158 tein that characterizes sporadic Creutzfeldt-Jakob disease can be found in certain brain regions of c
159 udy the white matter in sporadic Creutzfeldt-Jakob disease compared to healthy control subjects and t
160 Definite diagnosis of sporadic Creutzfeldt-Jakob disease in living patients remains a challenge.
164 ts appearance can mimic sporadic Creutzfeldt-Jakob disease on MRI and should be considered a differen
165 o improved detection of sporadic Creutzfeldt-Jakob disease prions in human nasal brushings and chroni
166 ely, the data show that sporadic Creutzfeldt-Jakob disease PrP(Sc) is not a single conformational ent
167 Cases diagnosed as sporadic Creutzfeldt-Jakob disease with atypical neuropathology were also rev
168 an idiopathic disorder (sporadic Creutzfeldt-Jakob disease) or can be acquired, as is the case for va
169 (15 of 15 with definite sporadic Creutzfeldt-Jakob disease, 13 of 14 with probable sporadic Creutzfel
170 13 of 14 with probable sporadic Creutzfeldt-Jakob disease, and 2 of 2 with inherited Creutzfeldt-Jak
171 amples from humans with sporadic Creutzfeldt-Jakob disease, as well as in rodents with experimental p
173 ALS, Alzheimer disease, sporadic Creutzfeldt-Jakob disease, Huntington disease-like syndrome, and oth
174 matter of patients with sporadic Creutzfeldt-Jakob disease, mainly in the left hemisphere, with a str
175 hin the white matter in sporadic Creutzfeldt-Jakob disease, suggesting possible primary involvement o
188 sed in the diagnosis of sporadic Creutzfeldt-Jakob disease; however, the characteristic waveforms do
191 h a mouse model of transmissible Creutzfeldt-Jakob disease (CJD), the ataxia of Tg(A116V) mice is mor
192 pathy (BSE) to humans as variant Creutzfeldt-Jakob disease (CJD) has affected over 100 individuals, a
195 prions from humans with variant Creutzfeldt-Jakob disease (CJD); on second passage in Tg(BoPrP) mice
196 autopsy-proved cases of variant Creutzfeldt-Jakob disease (n = 59) and compare these with cases of s
197 rP(Sc) molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably resulting fro
199 rodegenerative condition variant Creutzfeldt-Jakob disease (vCJD) and, based on recent human prevalen
200 a prototype blood-based variant Creutzfeldt-Jakob disease (vCJD) assay has sufficient sensitivity an
201 m, a deceased carrier of variant Creutzfeldt-Jakob disease (vCJD) can be followed up to quantify tran
204 o-person transmission of variant Creutzfeldt-Jakob disease (vCJD) has occurred through blood transfus
206 number of patients with variant Creutzfeldt-Jakob disease (vCJD) have been treated with intraventicu
207 rt a case of preclinical variant Creutzfeldt-Jakob disease (vCJD) in a patient who died from a non-ne
208 to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protect
214 clinical expression of, variant Creutzfeldt-Jakob disease (vCJD) is essential for future management
215 ns have been raised that variant Creutzfeldt-Jakob disease (vCJD) might be transmissible by blood tra
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
220 ern since the reports of variant Creutzfeldt-Jakob disease (vCJD) transmission through blood transfus
221 possible transmission of variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion has caused co
222 the risk of transmitting variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion have relied l
224 f patients affected with variant Creutzfeldt-Jakob disease (vCJD), a prion disease typically acquired
226 cribed in cases of human variant Creutzfeldt-Jakob disease (vCJD), experimental ovine bovine spongifo
227 orm encephalopathy (BSE)/variant Creutzfeldt-Jakob disease (vCJD), Nipah virus, several viral hemorrh
231 vidual can propagate the variant Creutzfeldt-Jakob disease agent and that the infectious agent can be
232 at the properties of the variant Creutzfeldt-Jakob disease agent could change after transmission to t
234 umulates in the brain in variant Creutzfeldt-Jakob disease also contains a minority type 1 component.
235 -mortem samples, of both variant Creutzfeldt-Jakob disease and Alzheimer's disease patients, also sug
237 o subsequently developed variant Creutzfeldt-Jakob disease and an asymptomatic red cell transfusion r
238 e urine of patients with variant Creutzfeldt-Jakob disease and had the typical electrophoretic profil
239 ained from patients with variant Creutzfeldt-Jakob disease and in none of the 224 urine samples obtai
240 on of 10% (wt/vol) human variant Creutzfeldt-Jakob disease brain homogenate, with >3,800-fold binding
243 against transmission of variant Creutzfeldt-Jakob disease by transfusion of domestic blood or red bl
244 Recent evidence that variant Creutzfeldt-Jakob disease can be transmitted by transfusion of red c
245 were followed-up in six variant Creutzfeldt-Jakob disease cases with 9.4 T high-resolution magnetiza
247 ained from patients with variant Creutzfeldt-Jakob disease contained minute quantities of PrP(Sc).
248 train characteristics of variant Creutzfeldt-Jakob disease had been modified by the host genotype, sp
249 The recent emergence of variant Creutzfeldt-Jakob disease has led to major public health concerns, a
250 The outbreak of new variant Creutzfeldt-Jakob disease has raised the specter of a potentially la
252 te and probable cases of variant Creutzfeldt-Jakob disease have been methionine homozygotes (MM).
253 4, a subclinical case of variant Creutzfeldt-Jakob disease in a PRNP 129 methionine/valine heterozygo
256 t numbers of subclinical variant Creutzfeldt-Jakob disease individuals in at least the United Kingdom
257 n, the identification of variant Creutzfeldt-Jakob disease infection in a PRNP 129 methionine/valine
258 idence of any death from variant Creutzfeldt-Jakob disease or from conditions that could be confused
259 ents for transmission of variant Creutzfeldt-Jakob disease predicted that leukocyte reduction would b
260 Evidence suggests that variant Creutzfeldt-Jakob disease prions circulate in body fluids from peopl
262 hus we have demonstrated variant Creutzfeldt-Jakob disease strain properties are not affected by tran
263 found in all 21 cases of variant Creutzfeldt-Jakob disease tested, irrespective of brain region exami
266 consistent with those of variant Creutzfeldt-Jakob disease transmitted to 129 methionine/methionine i
268 ypes was maintained when variant Creutzfeldt-Jakob disease was transmitted to wild-type mice and was
269 d animals, one of which (variant Creutzfeldt-Jakob disease) is known to be a zoonotic form of the cat
270 ncephalopathies, such as variant Creutzfeldt-Jakob disease, are believed to result from infectious fo
271 is a defining feature of variant Creutzfeldt-Jakob disease, but that the biochemical isoform of PrP(S
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 e urine of patients with variant Creutzfeldt-Jakob disease, we used the protein misfolding cyclic amp
277 f mid-2016, 231 cases of variant Creutzfeldt-Jakob disease-the human form of a prion disease of cattl
288 ations are applicable to variant Creutzfeldt-Jakob in humans then a method for rendering human red ce
289 probable transmission of variant Creutzfeldt-Jakob infectivity by transfusion of red cell preparation
290 anding of the risks of (variant) Creutzfeldt-Jakob disease transmission via dental practice, and whet
291 successfully propagated various Creutzfeldt-Jakob disease (CJD) isolates (sporadic, variant, and iat
292 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