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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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