[epidemiology of human prion diseases].prions(proteinaceous infectious particles) are responsible to subacute spongiform encephalopathies(sse) in man and animals. recent outbreak of bovine sse(bse), or mad cow disease in uk provoked concerns on its possible human hazards. a statement of the british government in march 1996 upset the world, which was based on 10 cases of "new variant" form of creutzfelds-jakob disease(cjd). prion diseases in animals are often epizootic and may be spread to different species through various routes incl ...19979103905
the spectrum of transmissible spongiform encephalopathies.since the first description by a.m. jakob and h.g. creutzfeldt, five human diseases have been identified as transmissible spongiform encephalopathies (tse). the disease bearing these authors' name, creutzfeldt-jakob disease (cjd) occurs sporadically, may be transmitted and has a genetic basis in 10-15% of all cases. genetic diseases are the gerstmann-sträussler-scheinker syndrome and fatal familial insomnia. the latest form of cjd in humans, variant cjd (vcjd), was first described in 1996 and ma ...19979450236
the impact of creutzfeldt-jakob disease and variant creutzfeldt-jakob disease on plasma safety.although the true risk of transmitting (classical) creutzfeld-jakob disease (cjd) and variant cjd (vcjd) via transfusion is likely very minimal, a review of prions and the impact of these associated prion diseases is timely because of their current effect on safety policies in the blood-plasma industry. various types of human and animal prion diseases are outlined and reviewed, with emphasis on the importance of cross-species transmission as is relevant for vcjd. review of the prion theory focus ...200111441420
transmissible spongiform encephalopathies in australia.the australian national creutzfeldt-jakob disease registry (ancjdr) commenced surveillance in september 1993 as part of the commonwealth's response to 4 cases of pituitary hormone (gonadotrophin)-associated creutzfeldt-jakob disease (cjd). with the passage of time, the registry has become responsible for ascertaining all human transmissible spongiform encephalopathies (tse; also known as prion diseases) within australia since 1970. included in the spectrum of diseases monitored are classical (sp ...200111806657
variant creutzfeldt-jakob disease: an unfolding epidemic of misfolded proteins.variant creutzfeldt-jakob disease (vcjd) is an emerging infectious disease believed to be the human manifestation of bovine spongiform encephalopathy (bse). variant cjd belongs to a family of human and animal diseases called transmissible spongiform encephalopathies (tse). the pathogenesis of tse is not fully understood, but a modified form of a normal cellular protein plays a central role. current measures to control vcjd aim to prevent transmission of the infectious agent from animals to human ...200212410862
neuronal and astrocytic responses involving the serotonergic system in human spongiform encephalopathies.the relationships between the degree of cortical prion protein (prp) deposition, tissue vacuolation and astrocytosis were studied in the frontal cortex of 27 cases of human spongiform encephalopathy, encompassing 13 cases of sporadic creutzfeldt-jakob disease (scjd), four cases of familial cjd (fcjd) (one owing to e200k mutation, one owing to 144 bp insertion, one owing to p102l mutation and one owing to a117v mutation), five cases of iatrogenic cjd (icjd) owing to growth hormone therapy and fiv ...200314507340
prion disease: possible implications for oral health care.prion diseases are a group of rare fatal neurodegenerative disorders in humans and animals that are histopathologically characterized by spongiform change within the central nervous system.200314664268
[comments on present-day spread and epidemiology of bse and prion diseases].prion diseases of animals and man are neurological diseases with amyloidal deposition of the respective proteins. as to prion disease, the cellular prion protein is in its abnormal isoform(s) an essential component of prion protein aggregates found in affected tissue. in contrast to all neurodegenerative diseases like morbus alzheimer or huntington's disease, prion diseases are transmissible. therefore, prion diseases were designated transmissible spongiform encephalopathies (tse). the diseases ...200414770333
creutzfeldt-jakob disease in ireland: epidemiological aspects 1980-2002.surveillance for creutzfeldt-jakob disease (cjd) has been carried out in the republic of ireland since 1980. initial surveillance was passive and based on consented autopsy confirmation of cjd in patients in whom there was a high index of clinical suspicion. since 1999, an active surveillance programme involving formal notification of all suspect cjd cases has been in place. the annual mortality rate has increased from 0.34 cases/million in 1980 to 1.27 cases/million in 2001. in all, 29 cases ha ...200414988606
biochemical fingerprints of prion diseases: scrapie prion protein in human prion diseases that share prion genotype and type.the phenotype of human prion diseases is influenced by the prion protein (prp) genotype as determined by the methionine (m)/valine (v) polymorphism at codon 129, the scrapie prp (prpsc) type and the etiology. to gain further insight into the mechanisms of phenotype determination, we compared two-dimensional immunoblot profiles of detergent insoluble and proteinase k-resistant prp species in a type of sporadic creutzfeldt-jakob disease (scjdmm2), variant cjd (vcjd) and sporadic fatal insomnia (sf ...200515606903
prion biology in transfusion medicine: implications for lab testing.although eerily silent for many years after the recognition of scrapie in 1759, tses remained present within the genome of some mammals. not since the mid-1950s when dr. carleton gadjusek visited the fore indians of new guinea to study kuru, however, has there been a more frenetic interest by governmental investigators. certainly, the u.k. experience has heralded a renewed interest in tses due to the notoriety associated with younger subjects succumbing to a variant cjd traced to the ingestion o ...200516265819
clinical implications of emerging pathogens in haemophilia: the variant creutzfeldt-jakob disease experience.the impact of variant creutzfeldt-jakob disease (vcjd) on the clinical practice of haemophilia in the uk is coloured by the haemophilia community's experience of hepatitis c virus and human immunodeficiency virus (hiv) transmission via plasma-derived therapies in the 1980s, when the delay in recognizing and acting on the potential risks cost many patients their lives and left others to manage another chronic disease. this crisis prompted organisations such as the united kingdom haemophilia centr ...200616445813
prions in dentistry--what are they, should we be concerned, and what can we do?to briefly review the characteristics of prions, the risk of transmission and implications for infection control in dentistry.200616480606
variant cjd (vcjd) and bovine spongiform encephalopathy (bse): 10 and 20 years on: part 1.from 1986 more than 184,000 cattle in the uk and islands (of which >1,880 have been detected by active surveillance using rapid tests) and approaching 5,500 elsewhere have been confirmed with bse. the original 1988 ban on the use of ruminant-derived protein in ruminant feed has been upgraded and now prohibits the use of any processed animal protein in feed for any farmed food animal. as a result of rigorous enforcement this reinforced ban is now regarded as fully effective from 1 aug. 1996. reas ...200616823691
variant cjd (vcjd) and bovine spongiform encephalopathy (bse): 10 and 20 years on: part 2.up until february 2006, variant cjd (vcjd), the human disease associated with transmission of bse from cattle, has been confirmed in 160 patients resident in the uk and 28 elsewhere, some of whom have never visited the uk. cases have been reported in france (16 cases), ireland (3), usa (2), canada, italy, japan, the netherlands, portugal, saudi arabia and spain (1 each). the presumed main period of hazard for ingestion of the bse agent in bovine products in the uk is 1984-89, or perhaps up to 19 ...200616823692
clinical, neuropathological and immunohistochemical features of sporadic and variant forms of creutzfeldt-jakob disease in the squirrel monkey (saimiri sciureus).the squirrel monkey (saimiri sciureus) has been shown to be nearly as susceptible as the chimpanzee to experimentally induced creutzfeldt-jakob disease (cjd), and has been used extensively in diagnostic and pathogenetic studies. however, no information is available concerning the clinicopathological characteristics of different strains of human transmissible spongiform encephalopathy (tse) in this species, in particular, strains of sporadic and variant cjd (scjd and vcjd, respectively). brain ho ...200717251588
current concepts in human prion protein (prp) misfolding, prnp gene polymorphisms and their contribution to creutzfeldt-jakob disease (cjd).transmissible spongiform encephalopathies are a group of neural degenerative diseases that may be infectious, sporadic, or hereditary and are associated with an abnormally folded prion protein. unfortunately at the current time it is not at all clear what the normal structure of the prion protein actually is or how it is toxic to cells. extensive research on prion diseases has led to a dramatic increase in understanding of the pathogenesis of prion disorders, which will hopefully lead to the dev ...200717616941
no evidence for association between tau gene haplotypic variants and susceptibility to creutzfeldt-jakob disease.a polymorphism at codon 129 of the prion protein gene (prnp) is the only well-known genetic risk factor for creutzfeldt-jakob disease (cjd). however, there is increasing evidence that other loci outside the prnp open reading frame might play a role in cjd aetiology as well.200718072964
tubulovesicular structures are a consistent (and unexplained) finding in the brains of humans with prion diseases.creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker disease (gss) and fatal familial insomnia (ffi) are slow neurodegenerative disorders classified as transmissible spongiform encephalopathies (tses) or prion diseases, which appear in sporadic, hereditary or environmentally acquired forms. tubulovesicular structures (tvs) are ultrastructural particles of unknown origin and chemical composition found in the brains of both animal and human forms of transmissible spongiform encephalopat ...200818164506
kuru: its ramifications after fifty years.kuru was the first human neurodegenerative disease in the group of transmissible spongiform encephalopathies, prion diseases or, in the past, slow unconventional virus diseases. it was reported to western medicine in 1957 by gajdusek and zigas. kuru was spread by endocannibalism and because of this the ratio of affected women and children to men was excessive. the hallmark of kuru neuropathology is the amyloid plaque. we may speculate what would happen if kuru had not been discovered or did not ...200918606515
hippocampal bursts caused by changes in nmda receptor-dependent excitation in a mouse model of variant cjd.prion diseases are heterogeneous in clinical presentation, suggesting that different prion diseases have distinct pathophysiological changes. to understand the pathophysiology specific to variant creutzfeldt-jakob disease (vcjd), in vitro electrophysiological studies were performed in a mouse model in which human-derived vcjd prions were transmitted to transgenic mice expressing human instead of murine prion protein. paired-pulse stimulation of the schaffer collaterals evoked hypersynchronous bu ...200818638557
prion removal effect of a specific affinity ligand introduced into the manufacturing process of the pharmaceutical quality solvent/detergent (s/d)-treated plasma octaplaslg.a new chromatographic step for the selective binding of abnormal prion protein (prp(sc)) was developed, and optimization for prp(sc) capture was achieved by binding to an affinity ligand attached to synthetic resin particles. this step was implemented into the manufacturing process of the solvent/detergent (s/d)-treated biopharmaceutical quality plasma octaplas to further improve the safety margin in terms of risk for variant creutzfeldt-jakob disease (vcjd) transmission.200919548963
[risk factors for sporadic creutzfeldt-jakob disease].sporadic creutzfeldt-jakob disease (scjd) is the most common form of human transmissible spongiform encephalopathies (prion disease), but its cause has not been fully elucidated. according to its biochemical properties prion protein is resistant to routine sterilisation methods. thus, invasive medical procedures could be involved in the genesis of the disease. present knowledge about iatrogenic routes of transmission, oral infection and transmission via blood products in variant cjd (vcjd) under ...200919551608
human prion diseases in the united states.prion diseases are a family of rare, progressive, neurodegenerative disorders that affect humans and animals. the most common form of human prion disease, creutzfeldt-jakob disease (cjd), occurs worldwide. variant cjd (vcjd), a recently emerged human prion disease, is a zoonotic foodborne disorder that occurs almost exclusively in countries with outbreaks of bovine spongiform encephalopathy. this study describes the occurrence and epidemiology of cjd and vcjd in the united states.201020049325
the prion diseases.the prion diseases are a family of rare neurodegenerative disorders that result from the accumulation of a misfolded isoform of the prion protein (prp), a normal constituent of the neuronal membrane. five subtypes constitute the known human prion diseases; kuru, creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker syndrome (gss), fatal insomnia (fi), and variant cjd (vcjd). these subtypes are distinguished, in part, by their clinical phenotype, but primarily by their associated brain ...201020938044
Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP.Prion diseases are fatal neurodegenerative diseases of humans and animals caused by the misfolding and aggregation of prion protein (PrP). Mammalian prion diseases are under strong genetic control but few risk factors are known aside from the PrP gene locus (PRNP). No genome-wide association study (GWAS) has been done aside from a small sample of variant Creutzfeldt-Jakob disease. We conducted GWAS of sporadic CJD, variant CJD, iatrogenic CJD, inherited prion disease, kuru and resistance to kuru ...201122210626
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