1175:
totally surrounded by optical fibres to capture all light emitted once the dye is excited using a laser. The technique allowed detection of PrP after many fewer cycles of conversion than others have achieved, substantially reducing the possibility of artefacts, as well as speeding up the assay. The researchers also tested their method on blood samples from apparently healthy sheep that went on to develop scrapie. The animals' brains were analysed once any symptoms became apparent. The researchers could therefore compare results from brain tissue and blood taken once the animals exhibited symptoms of the diseases, with blood obtained earlier in the animals' lives, and from uninfected animals. The results showed very clearly that PrP could be detected in the blood of animals long before the symptoms appeared.
86:
130:
1204:(from use of contaminated surgical equipment). This was before equipment sterilization was required in 1976, and there have been no other iatrogenic cases since then. In order to prevent the spread of infection, the World Health Organization created a guide to tell health care workers what to do when CJD appears and how to dispose of contaminated equipment. The Centers for Disease Control and Prevention (CDC) have been keeping surveillance on CJD cases, particularly by looking at death certificate information.
1166:
has started. At present, there is virtually no way to detect PrP reliably except by examining the brain using neuropathological and immunohistochemical methods after death. Accumulation of the abnormally folded PrP form of the PrP protein is a characteristic of the disease, but it is present at very low levels in easily accessible body fluids like blood or urine. Researchers have tried to develop methods to measure PrP, but there are still no fully accepted methods for use in materials such as blood.
1208:
deer and elk spread in northeastern
Colorado, southeastern Wyoming and western Nebraska. It was also discovered that CWD may have been present in a proportion of free-ranging animals decades before the initial recognition. In the United States, the discovery of CWD raised concerns about the transmission of this prion disease to humans. Many apparent cases of CJD were suspected transmission of CWD, however the evidence was lacking and not convincing.
1212:
human consumption of these infected cattle caused an outbreak of the human form CJD. There was a dramatic decline in BSE when feeding bans were put in place. On May 20, 2003, the first case of BSE was confirmed in North
America. The source could not be clearly identified, but researchers suspect it came from imported BSE-infected cow meat. In the United States, the USDA created safeguards to minimize the risk of BSE exposure to humans.
997:
25:
1257:'s discovery that Kuru was transmitted by cannibalism accompanied by the finding of scrapie-like lesions in the brains of Kuru victims strongly suggested an infectious basis to TSE. A paradigm shift to a non-nucleic infectious entity was required when the results were validated with an explanation of how a
1195:
Transmissible spongiform encephalopathies (TSE) are very rare but can reach epidemic proportions. It is very hard to map the spread of the disease due to the difficulty of identifying individual strains of the prions. This means that, if animals at one farm begin to show the disease after an outbreak
1207:
Chronic wasting disease (CWD) is a prion disease found in North
America in deer and elk. The first case was identified as a fatal wasting syndrome in the 1960s. It was then recognized as a transmissible spongiform encephalopathy in 1978. Surveillance studies showed the endemic of CWD in free-ranging
889:
Early neuropathological reports on human prion diseases suffered from a confusion of nomenclature, in which the significance of the diagnostic feature of spongiform change was occasionally overlooked. The subsequent demonstration that human prion diseases were transmissible reinforced the importance
1215:
Variant
Creutzfeldt-Jakob disease (vCJD) was discovered in 1996 in England. There is strong evidence to suggest that vCJD was caused by the same prion as bovine spongiform encephalopathy. A total of 231 cases of vCJD have been reported since it was first discovered. These cases have been found in a
1165:
There continues to be a very practical problem with diagnosis of prion diseases, including BSE and CJD. They have an incubation period of months to decades during which there are no symptoms, even though the pathway of converting the normal brain PrP protein into the toxic, disease-related PrP form
1211:
In the 1980s and 1990s, bovine spongiform encephalopathy (BSE or "mad cow disease") spread in cattle at an epidemic rate. The total estimated number of cattle infected was approximately 750,000 between 1980 and 1996. This occurred because the cattle were fed processed remains of other cattle. Then
1064:
cause prion disease. Familial forms of prion disease are caused by inherited mutations in the PRNP gene. Only a small percentage of all cases of prion disease run in families, however. Most cases of prion disease are sporadic, which means they occur in people without any known risk factors or gene
1174:
and some specific antibodies against PrP. After amplifying and then concentrating any PrP, the samples are labelled with a fluorescent dye using an antibody for specificity and then finally loaded into a micro-capillary tube. This tube is placed in a specially constructed apparatus so that it is
1131:
While not containing a nucleic acid genome, prions may be composed of more than just a protein. Purified PrP appears unable to convert to the infectious PrP form, unless other components are added, such as RNA and lipids. These other components, termed cofactors, may form part of the infectious
1199:
Classic
Creutzfeldt-Jakob disease (CJD) was discovered in 1920. It occurs sporadically over the world but is very rare. It affects about one person per million each year. Typically, the cause is unknown for these cases. It has been found to be passed on genetically in some cases. 250 patients
1240:
was discussed in the
British House of Commons and may have been present in Britain for some time before that. Although there were unsupported claims in 1759 that the disease was contagious, in general it was thought to be due to inbreeding and countermeasures appeared to be successful.
2996:
Hope J, Reekie LJ, Hunter N, Multhaup G, Beyreuther K, White H, Scott AC, Stack MJ, Dawson M, Wells GA.; Reekie; Hunter; Multhaup; Beyreuther; White; Scott; Stack; Dawson; et al. (Nov 1988). "Fibrils from brains of cows with new cattle disease contain scrapie-associated protein".
2047:
Sakudo, Akikazu; Lee, Deug-chan; Saeki, Keiichi; Nakamura, Yuko; Inoue, Keiichi; Matsumoto, Yoshitsugu; Itohara, Shigeyoshi; Onodera, Takashi (2003). "Impairment of superoxide dismutase activation by N-terminally truncated prion protein (PrP) in PrP-deficient neuronal cell line".
371:
and familial spongiform encephalopathy. Creutzfeldt-Jakob disease itself has four main forms, the sporadic (sCJD), the hereditary/familial (fCJD), the iatrogenic (iCJD) and the variant form (vCJD). These conditions form a spectrum of diseases with overlapping signs and symptoms.
3062:
Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, Poser S, Pocchiari M, Hofman A, Smith PG.; Ironside; Zeidler; Cousens; Estibeiro; Alperovitch; Poser; Pocchiari; Hofman; Smith (April 1996). "A new variant of
Creutzfeldt–Jakob disease in the UK".
1083:
Familial forms of prion disease are inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person inherits the altered gene from one affected parent.
1216:
total of 12 countries with 178 in the United
Kingdom, 27 in France, five in Spain, four in Ireland, four in the United States, three in the Netherlands, three in Italy, two in Portugal, two in Canada, and one each in Japan, Saudi Arabia, and Taiwan.
1678:
Nitrini R, Rosemberg S, Passos-Bueno MR, da Silva LS, Iughetti P, Papadopoulos M, Carrilho PM, Caramelli P, Albrecht S, Zatz M, LeBlanc A (August 1997). "Familial spongiform encephalopathy associated with a novel prion protein gene mutation".
1520:
Brown P, Preece M, Brandel JP, Sato T, McShane L, Zerr I, Fletcher A, Will RG, Pocchiari M, Cashman NR, d'Aignaux JH, Cervenakova L, Fradkin J, Schonberger LB, Collins SJ (2000). "Iatrogenic
Creutzfeldt–Jakob disease at the millennium".
1835:
Haybaeck, Johannes; Heikenwalder, Mathias; Klevenz, Britta; Schwarz, Petra; Margalith, Ilan; Bridel, Claire; Mertz, Kirsten; Zirdum, Elizabeta; Petsch, Benjamin; Fuchs, Thomas J.; Stitz, Lothar; Aguzzi, Adriano (January 13, 2011).
466:
procedures such as boiling or irradiating materials fail to render prions non-infective. However, treatment with strong, almost undiluted bleach and/or sodium hydroxide, or heating to a minimum of 134 °C, does destroy prions.
461:
Prions cannot be transmitted through the air, through touching, or most other forms of casual contact. However, they may be transmitted through contact with infected tissue, body fluids, or contaminated medical instruments. Normal
1140:
This hypothesis postulates that a yet undiscovered infectious viral agent is the cause of the disease. Although this was once the leading hypothesis, it is now a minority view. Evidence for this hypothesis is as follows:
1634:
1265:
in the
British cattle herd heightened fear of transmission to humans and reinforced the belief in the infectious nature of TSE. This was confirmed with the identification of a Kuru-like disease, called new variant
1079:
in this gene cause cells to produce an abnormal form of the prion protein, known as PrP. This abnormal protein builds up in the brain and destroys nerve cells, resulting in the signs and symptoms of prion disease.
1087:
In some people, familial forms of prion disease are caused by a new mutation in the PRNP gene. Although such people most likely do not have an affected parent, they can pass the genetic change to their children.
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3758:
3739:
1169:
In 2010, a team from New York described detection of PrP even when initially present at only one part in a hundred billion (10) in brain tissue. The method combines amplification with a novel technology called
1114:
PrP depletion in the neural system of mice with established neuroinvasive prion infection reverses early spongeosis and behavioural deficits, halts further disease progression and increases life-span
1792:
Collins S, McLean CA, Masters CL (2001). "Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia, and kuru: a review of these less common human transmissible spongiform encephalopathies".
1196:
on a nearby farm, it is very difficult to determine whether it is the same strain affecting both herds—suggesting transmission—or if the second outbreak came from a completely different source.
978:
smokers has not yet been identified. This, combined with the highly variable nature of prion disease pathology, is why a prion disease cannot be diagnosed based solely on a patient's symptoms.
851:
plaque formation. These features are shared with prion diseases in animals, and the recognition of these similarities prompted the first attempts to transmit a human prion disease (kuru) to a
1065:
mutations. In rare circumstances, prion diseases also can be transmitted by exposure to prion-contaminated tissues or other biological materials obtained from individuals with prion disease.
1072:(PrP). Under normal circumstances, this protein may be involved in transporting copper into cells. The protein may also be involved in protecting brain cells and helping them communicate.
2236:
Sakudo A; Lee DC; Saeki K; et al. (August 2003). "Impairment of superoxide dismutase activation by N-terminally truncated prion protein (PrP) in PrP-deficient neuronal cell line".
835:
The degenerative tissue damage caused by human prion diseases (CJD, GSS, and kuru) is characterised by four features: spongiform change (the presence of many small holes), the death of
913:" sterilization process used for most surgical instruments). It is also believed that dietary consumption of affected animals can cause prions to accumulate slowly, especially when
2532:"Transmissible Spongiform Encephalopathies (TSEs), also known as prion diseases | Anses - Agence nationale de sécurité sanitaire de l'alimentation, de l'environnement et du travail"
454:, a practice now banned in many countries. In turn, consumption (by humans) of bovine-derived foodstuff which contained prion-contaminated tissues resulted in an outbreak of the
893:
Prions appear to be most infectious when in direct contact with affected tissues. For example, Creutzfeldt–Jakob disease has been transmitted to patients taking injections of
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protein might transmit spongiform encephalopathy. Not until 1988 was the neuropathology of spongiform encephalopathy properly described in cows. The alarming amplification of
2774:
3061:
3710:
1096:
Protein could be the infectious agent, inducing its own replication by causing conformational change of normal cellular PrP into PrP. Evidence for this hypothesis:
446:. Transmission occurs when healthy animals consume tainted tissues from others with the disease. In the 1980s and 1990s, bovine spongiform encephalopathy spread in
1469:
Collinge, J; Sidle, KC; Meads, J; Ironside, J; Hill, AF (October 24, 1996). "Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD".
1018:
434:
means (e.g., blood transfusion). Most TSEs are sporadic and occur in an animal with no prion protein mutation. Inherited TSE occurs in animals carrying a rare
1278:
transplantation model that explains why cannibalism favours transmission, the search for a viral agent was, as of 2007, being continued in some laboratories.
859:
diagnosis of human prion diseases for many years, although it was recognized that these changes are enormously variable both from case to case and within the
1950:
Kriegstein AR; Shungu DC; Millar WS; et al. (1999). "Leukoencephalopathy and raised brain lactate from heroin vapor inhalation ("chasing the dragon")".
2995:
3850:
3348:
2271:
Mallucci G; Dickinson A; Lineham J; et al. (October 2003). "Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis".
1183:
There are currently no known ways to cure or prevent prion disease. Certain medications can slow down the progression of the disease. But ultimately,
356:
3219:
3106:
3048:
2982:
1722:
Jeffrey M, Goodbrand IA, Goodsir CM (1995). "Pathology of the transmissible spongiform encephalopathies with special emphasis on ultrastructure".
2091:
Collinge, John; Whittington, Miles A.; Sidle, Katie C. L.; Smith, Corinne J.; Palmer, Mark S.; Clarke, Anthony R.; Jefferys, John G. R. (1994).
1245:
between animals, until extraordinary measures were taken such as the intra-ocular injection of infected nervous tissue. No direct link between
233:
Contact with infected fluids, ingestion of infected flesh, having one or two parents that have the disease (in case of fatal familial insomnia)
2531:
1564:
Colle, JG; Bradley, R; Libersky, PP (2006). "Variant CJD (vCJD) and bovine spongiform encephalopathy (BSE): 10 and 20 years on: part 2".
890:
of spongiform change as a diagnostic feature, reflected in the use of the term "spongiform encephalopathy" for this group of disorders.
1453:
1171:
947:
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1307:
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1423:
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368:
183:
123:
974:(PSL)—which is a spongiform encephalopathy—is also probably not caused by a prion, although the adulterant that causes it among
2739:
3502:
3412:
3363:
760:
2148:"High titers of transmissible spongiform encephalopathy infectivity associated with extremely low levels of PrPSc in vivo"
1925:
3250:
This entry incorporates public domain text originally from the National Institute of Neurological Disorders and Stroke,
3121:
1610:"ICTVdB : the universal virus database of the International Committee on Taxonomy of Viruses - NLM Catalog - NCBI"
3582:
3459:
3257:
1044:
546:
455:
388:
199:
1026:
3632:
3464:
3439:
3162:"Cells infected with scrapie and Creutzfeldt–Jakob disease agents produce intracellular 25-nm virus-like particles"
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1271:
1262:
1233:
635:
608:
392:
380:
195:
159:
3444:
1380:
Chesebro, Bruce (2003-06-01). "Introduction to the transmissible spongiform encephalopathies or prion diseases".
1236:
records cases of a disease with similar characteristics in the 4th and 5th centuries AD. In 1755, an outbreak of
688:
207:
1341:
spp. from transmissible spongiform encephalopathy brains or ticks induce spongiform encephalopathy in ruminants"
855:
in 1966, followed by CJD in 1968 and GSS in 1981. These neuropathological features have formed the basis of the
3669:
3469:
1022:
936:
proteins in ruminant feed as a precaution against the spread of prion infection in cattle and other ruminants.
660:
203:
45:
3407:
3379:
3343:
3281:
3251:
2939:
Collins SJ, Lawson VA, Masters CL.; Lawson; Masters (Jan 2004). "Transmissible spongiform encephalopathies".
2481:
1655:
1267:
882:, confusion, or memory problems. In the later stages of the disease, patients have severe mental impairment (
741:
352:
344:. The disorders cause impairment of brain function which may result in memory loss, personality changes, and
102:
3752:
3296:
463:
2482:"Therapeutic Approaches for Prion Diseases | NIAID: National Institute of Allergy and Infectious Diseases"
866:
The clinical signs in humans vary, but commonly include personality changes, psychiatric problems such as
3612:
422:
material. Misfolded prion proteins carry the disease between individuals and cause deterioration of the
3156:
85:
929:, who used to consume their dead as a funerary ritual. Laws in developed countries now ban the use of
3679:
3495:
1428:
971:
167:
3788:
3358:
3122:"Sacred disease of our times: failure of the infectious disease model of spongiform encephalopathy"
2740:"Sacred disease of our times: failure of the infectious disease model of spongiform encephalopathy"
1254:
1249:
and human disease was suspected then or has been found since. TSE was first described in humans by
1007:
917:
or similar practices allow the proteins to accumulate over more than one generation. An example is
211:
129:
3454:
3289:
1595:
1201:
1011:
799:
571:
384:
360:
187:
163:
3254:
2669:
2625:
2581:"Surveillance for vCJD | Variant Creutzfeldt-Jakob Disease, Classic (CJD) | Prion Disease | CDC"
3648:
3627:
2457:
930:
860:
3748:
2507:
3622:
3607:
3213:
3100:
3042:
2976:
1757:
Collinge J (2001). "Prion diseases of humans and animals: their causes and molecular basis".
1448:
1232:
described a disease like TSE in cattle and sheep, which he believed also occurred in humans.
1184:
3488:
3173:
3006:
2905:
2896:
Gajdusek DC (Sep 1977). "Unconventional viruses and the origin and disappearance of kuru".
2852:
2649:
2605:
2335:
2280:
2104:
1993:
Chang YJ, Tsai CH, Chen CJ (1997). "Leukoencephalopathy after inhalation of heroin vapor".
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8:
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427:
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1482:
3653:
3589:
3545:
3196:
3161:
3088:
3030:
2964:
2878:
2816:
2791:
2718:"Risk for Travelers | Variant Creutzfeldt-Jakob Disease, Classic (CJD) | Prion Disease"
2696:
2358:
2323:
2304:
1975:
1907:
1899:
1864:
1837:
1817:
1704:
1609:
1546:
1502:
1434:
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451:
332:
infection. Mental and physical abilities deteriorate and many tiny holes appear in the
294:, are a group of progressive, incurable, and fatal conditions that are associated with
138:
3233:
3076:
2952:
2717:
2249:
2213:
2188:
2061:
939:
There exists evidence that prion diseases may be transmissible by the airborne route.
475:
Differences in shape between the different prion protein forms are poorly understood.
3799:
3599:
3530:
3201:
3133:
3080:
3022:
2956:
2921:
2882:
2870:
2821:
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2404:
2363:
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2002:
1967:
1888:
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1809:
1774:
1739:
1735:
1696:
1649:
1573:
1538:
1494:
1405:
1397:
1362:
1335:, Sanders DE, Forbes WA, Hagius SD, Walker JV, Henk WG, Enright FM, Elzer PH (2007).
914:
581:
443:
442:, which expresses prion proteins that contort by themselves into the disease-causing
430:
may be genetic, sporadic, or infectious via ingestion of infected foodstuffs and via
238:
118:
3828:
3782:
3092:
2968:
2580:
2557:"Infection Control | Creutzfeldt-Jakob Disease, Classic (CJD) | Prion Disease | CDC"
2308:
2204:
1821:
1708:
1550:
3577:
3191:
3181:
3160:, Yu ZX, Barquero N, Banquero N, Mullins B; Yu; Banquero; Mullins (February 2007).
3157:
3072:
3034:
3014:
2948:
2913:
2860:
2811:
2803:
2792:"1755 and all that: a historical primer of transmissible spongiform encephalopathy"
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2394:
2353:
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2200:
2159:
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1979:
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1801:
1770:
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1731:
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1486:
1389:
1352:
1332:
1132:
prion, or they may serve as catalysts for the replication of a protein-only prion.
926:
1591:
1274:. Although the infectious disease model of TSE has been questioned in favour of a
3793:
3535:
2458:"SOFIA: An Assay Platform for Ultrasensitive Detection of PrP in Brain and Blood"
1884:
1854:
898:
333:
98:
39:
3260:
1449:"Variant Creutzfeldt-Jakob disease > Relationship with BSE (Mad Cow Disease)"
3804:
3540:
3417:
3384:
3353:
3166:
Proceedings of the National Academy of Sciences of the United States of America
2938:
2328:
Proceedings of the National Academy of Sciences of the United States of America
1929:
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1401:
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922:
906:
3767:
3186:
2348:
2292:
1635:"Deux chercheurs algériens découvrent la maladie du "chameau fou" à Ouargla"
398:
Unlike other kinds of infectious disease, which are spread by agents with a
3705:
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2634:
2408:
2367:
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1971:
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1577:
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840:
670:
431:
106:
3084:
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2006:
1963:
1896:. If this is an intentional citation to a retracted paper, please replace
1743:
1700:
1692:
1534:
1498:
1357:
1336:
3511:
2925:
1393:
1229:
328:
243:
Currently there is no way to reliably detect prions except at post-mortem
228:
1102:
Infectivity titre correlates with PrP levels. However, this is disputed.
1148:
902:
844:
383:(BSE) in cattle – popularly known as "mad cow disease" – and
341:
322:. According to the most widespread hypothesis, they are transmitted by
3731:
2270:
1838:"Aerosols Transmit Prions to Immunocompetent and Immunodeficient Mice"
3823:
3695:
3018:
2116:
2020:
Koussa S, Zabad R, Rizk T, Tamraz J, Nasnas R, Chemaly R (2002). "".
1490:
1068:
The PRNP gene provides the instructions to make a protein called the
910:
875:
874:). Patients also may experience involuntary jerking movements called
856:
585:
264:
143:
Dementia, seizures, tremors, insomnia, psychosis, delirium, confusion
1677:
996:
921:, which reached epidemic proportions in the mid-20th century in the
1076:
951:
933:
883:
879:
589:
435:
411:
2865:
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1834:
1592:"Prion Disinfection Options - Biosafety & Occupational Health"
450:
in an epidemic fashion. This occurred because cattle were fed the
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causing it to appear like a sponge when brain tissue obtained at
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110:
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Early-20th-century experiments failed to show transmission of
3312:
2324:"Formation of native prions from minimal components in vitro"
2321:
2189:"Branched Polyamines Cure Prion-Infected Neuroblastoma Cells"
2146:
Barron RM; Campbell SL; King D; et al. (December 2007).
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523:
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Deleault NR, Harris BT, Rees JR, Supattapone S (June 2007).
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Supattapone S; Wille H; Uyechi L; et al. (April 2001).
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175:
2145:
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Traditional Chinese medicines derived from the human body
2789:
2093:"Prion protein is necessary for normal synaptic function"
1225:
641:
577:
403:
399:
1721:
1312:
National Institute of Neurological Disorders and Stroke
1187:
is currently the only option for infected individuals.
2046:
1637:. 2018-05-09. Archived from the original on 2018-06-17
1519:
2647:
2603:
2235:
2019:
326:, though some other data suggest an involvement of a
3721:
1791:
1563:
414:), the infectious agent in TSEs is believed to be a
2238:
Biochemical and Biophysical Research Communications
2050:
Biochemical and Biophysical Research Communications
870:, lack of coordination, and/or an unsteady gait (
3842:
97:that appear as holes in tissue sections) in the
847:due to the destruction of nearby neurons), and
3150:
1154:Strain variation of different isolates of PrP.
3496:
3297:
2790:Brown P, Bradley R; Bradley (December 1998).
2315:
1992:
1120:
71:Transmissible spongiform encephalopathy (TSE)
3218:: CS1 maint: multiple names: authors list (
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2650:"The Public Health Impact of Prion Diseases"
2606:"The Public Health Impact of Prion Diseases"
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1325:
93:Micrograph showing spongiform degeneration (
38:"Cause" section is in fairly bad shape with
2895:
2838:
2695:. World Health Organization. Archived from
2670:10.1146/annurev.publhealth.26.021304.144536
2626:10.1146/annurev.publhealth.26.021304.144536
1986:
1308:"Transmissible Spongiform Encephalopathies"
1025:. Unsourced material may be challenged and
3503:
3489:
3304:
3290:
1910:|...|intentional=yes}}
1756:
1668:Believed to be identical to the BSE prion.
1454:Centers for Disease Control and Prevention
1200:contracted the disease through iatrogenic
1172:Surround Optical Fiber Immunoassay (SOFIA)
1091:
972:Progressive Spongiform Leukoencephalopathy
128:
84:
34:needs attention from an expert in Medicine
3851:Transmissible spongiform encephalopathies
3195:
3185:
3119:
2864:
2841:"It's Jakob's disease, not Creutzfeldt's"
2815:
2737:
2668:
2624:
2398:
2357:
2347:
2212:
2163:
1863:
1853:
1356:
1045:Learn how and when to remove this message
946:are caused by prions, as in the cases of
905:allografts and from instruments used for
886:) and lose the ability to move or speak.
456:variant form of Creutzfeldt–Jakob disease
426:. TSEs are unique diseases in that their
389:variant form of Creutzfeldt–Jakob disease
284:Transmissible spongiform encephalopathies
16:Group of brain diseases induced by prions
2380:
1379:
3390:Variably protease-sensitive prionopathy
3349:Gerstmann–Sträussler–Scheinker syndrome
3317:transmissible spongiform encephalopathy
3273:Transmissible spongiform encephalopathy
1127:Protein misfolding cyclic amplification
909:(Brown, 2000) (prions can survive the "
780:Gerstmann-Sträussler-Scheinker syndrome
369:variably protease-sensitive prionopathy
357:Gerstmann–Sträussler–Scheinker syndrome
192:Gerstmann-Sträussler-Scheinker syndrome
184:variably protease-sensitive prionopathy
3843:
48:may be able to help recruit an expert.
3484:
3285:
830:
367:, as well as the recently discovered
1602:
1300:
1023:adding citations to reliable sources
990:
843:(abnormal increase in the number of
613:commonly known as "mad cow disease"
18:
2693:"Variant Creutzfeldt-Jakob disease"
2505:
2152:The Journal of Biological Chemistry
1424:"Variant Creutzfeldt-Jakob disease"
1147:Incubation time is comparable to a
1135:
818:Familial spongiform encephalopathy
391:in humans is caused by exposure to
13:
2432:: 195. August 2010. Archived from
1108:Denaturing PrP removes infectivity
479:Known spongiform encephalopathies
375:TSEs in non-human mammals include
14:
3862:
3460:Transmissible mink encephalopathy
3266:
3258:U.S. National Library of Medicine
761:Variant Creutzfeldt–Jakob disease
547:Transmissible mink encephalopathy
470:
452:processed remains of other cattle
200:transmissible mink encephalopathy
3465:Feline spongiform encephalopathy
3440:Bovine spongiform encephalopathy
2779:Publius Flavius Vegetius Renatus
1234:Publius Flavius Vegetius Renatus
1111:PrP-null mice cannot be infected
995:
636:Feline spongiform encephalopathy
609:Bovine spongiform encephalopathy
418:, thus being composed solely of
393:bovine spongiform encephalopathy
381:bovine spongiform encephalopathy
196:feline spongiform encephalopathy
160:Bovine spongiform encephalopathy
23:
3510:
3445:Camel spongiform encephalopathy
3226:
3113:
3055:
2989:
2932:
2889:
2832:
2783:
2768:
2731:
2710:
2685:
2641:
2597:
2573:
2549:
2524:
2499:
2474:
2463:. SUNY Downstate Medical Center
2450:
2415:
2374:
2264:
2229:
2205:10.1128/JVI.75.7.3453-3461.2001
2180:
2139:
2084:
2040:
1918:
1828:
1785:
1750:
1715:
1671:
1662:
1627:
1433:. February 2012. Archived from
1345:Journal of Medical Microbiology
1190:
966:(caused by a deficiency of the
689:Camel spongiform encephalopathy
208:camel spongiform encephalopathy
3470:Exotic ungulate encephalopathy
2657:Annual Review of Public Health
2648:Belay and Schonberger (2005).
2613:Annual Review of Public Health
2604:Belay and Schonberger (2005).
1771:10.1146/annurev.neuro.24.1.519
1584:
1557:
1513:
1462:
1441:
1416:
1373:
661:Exotic ungulate encephalopathy
204:exotic ungulate encephalopathy
1:
3252:National Institutes of Health
3077:10.1016/S0140-6736(96)91412-9
2953:10.1016/S0140-6736(03)15171-9
2250:10.1016/S0006-291X(03)01459-1
2062:10.1016/s0006-291x(03)01459-1
1293:
346:abnormal or impaired movement
101:of a patient who had died of
1885:10.1371/journal.ppat.1012396
1855:10.1371/journal.ppat.1001257
1736:10.1016/0968-4328(95)00004-N
1178:
1160:
7:
3364:Huntington's disease-like 1
2775:Digesta Artis Mulomedicinae
2423:"Detecting Prions in Blood"
1281:
986:
387:(CWD) in deer and elk. The
176:Huntington's disease-like 1
36:. The specific problem is:
10:
3867:
3120:McAlister, V (June 2005).
2738:McAlister, V (June 2005).
1219:
1124:
1121:Multi-component hypothesis
3814:
3725:
3688:
3662:
3641:
3598:
3518:
3430:
3398:
3372:
3332:
3323:
2808:10.1136/bmj.317.7174.1688
1654:: CS1 maint: unfit URL (
1429:World Health Organization
1268:Creutzfeldt–Jakob disease
742:Creutzfeldt–Jakob disease
721:
708:
506:
353:Creutzfeldt–Jakob disease
273:
263:
255:
247:
237:
227:
217:
168:Creutzfeldt-Jakob disease
155:
147:
137:
117:
103:Creutzfeldt–Jakob disease
92:
83:
75:
70:
3359:PrP systemic amyloidosis
2839:Katscher F. (May 1998).
2387:British Medical Bulletin
1382:British Medical Bulletin
1255:Daniel Carleton Gajdusek
981:
458:in the 1990s and 2000s.
348:which worsen over time.
212:PrP systemic amyloidosis
3455:Chronic wasting disease
3385:Sporadic fatal insomnia
3354:Fatal familial insomnia
3187:10.1073/pnas.0610999104
2349:10.1073/pnas.0702662104
2293:10.1126/science.1090187
1596:University of Minnesota
1270:, in humans exposed to
1105:PrP is an isomer of PrP
1092:Protein-only hypothesis
970:galactosylceramidase).
962:protein activity), and
800:Fatal familial insomnia
572:Chronic wasting disease
385:chronic wasting disease
361:fatal familial insomnia
351:TSEs of humans include
188:chronic wasting disease
164:Fatal familial insomnia
3234:"Infectious Particles"
2918:10.1126/science.142303
2383:"TSE strain variation"
2165:10.1074/jbc.M704329200
1806:10.1054/jocn.2001.0919
1456:. Retrieved 2017-04-25
1431:. Retrieved 2017-04-25
878:, unusual sensations,
861:central nervous system
2512:National Prion Clinic
1995:J. Formos. Med. Assoc
1964:10.1212/WNL.53.8.1765
1902:|...}}
1879:(Retracted, see
1693:10.1002/ana.410420203
1535:10.1212/WNL.55.8.1075
1437:on December 20, 2002.
1358:10.1099/jmm.0.47159-0
897:harvested from human
863:in individual cases.
2699:on December 20, 2002
2022:Rev. Neurol. (Paris)
1614:www.ncbi.nlm.nih.gov
1566:Folia Neuropatholica
1019:improve this section
958:(caused by abnormal
340:is examined under a
46:WikiProject Medicine
3551:Kleptopharmacophagy
3178:2007PNAS..104.1965M
3011:1988Natur.336..390H
2910:1977Sci...197..943C
2857:1998Natur.393Q..11K
2430:Microbiology Today.
2400:10.1093/bmb/66.1.99
2340:2007PNAS..104.9741D
2285:2003Sci...302..871M
2193:Journal of Virology
2109:1994Natur.370..295C
1932:on November 1, 2004
1681:Annals of Neurology
1598:. 17 November 2017.
1483:1996Natur.383..685C
1458:. 10 February 2015.
1224:In the 5th century
480:
124:Infectious diseases
3815:External resources
2538:. 18 February 2013
2506:UCL (2021-02-02).
1394:10.1093/bmb/66.1.1
1251:Alfons Maria Jakob
942:Note that not all
831:Signs and symptoms
478:
3838:
3837:
3719:
3718:
3478:
3477:
3426:
3425:
2802:(7174): 1688–92.
2508:"Drug treatments"
2486:www.niaid.nih.gov
2381:Bruce ME (2003).
2279:(5646): 871–874.
2103:(6487): 295–297.
1759:Annu Rev Neurosci
1477:(6602): 685–690.
1057:Mutations in the
1055:
1054:
1047:
828:
827:
796:90.001.0.01.010.
776:90.001.0.01.009.
738:90.001.0.01.008.
714:90.001.0.01.007.
657:90.001.0.01.006.
632:90.001.0.01.005.
605:90.001.0.01.004.
582:white-tailed deer
568:90.001.0.01.003.
543:90.001.0.01.002.
515:90.001.0.01.001.
290:), also known as
281:
280:
239:Diagnostic method
151:Months to decades
109:, scale bar = 30
65:Medical condition
63:
62:
3858:
3723:
3722:
3689:Related concepts
3505:
3498:
3491:
3482:
3481:
3433:in other animals
3330:
3329:
3306:
3299:
3292:
3283:
3282:
3242:
3241:
3230:
3224:
3223:
3217:
3209:
3199:
3189:
3154:
3148:
3147:
3145:
3144:
3117:
3111:
3110:
3104:
3096:
3059:
3053:
3052:
3046:
3038:
3019:10.1038/336390a0
2993:
2987:
2986:
2980:
2972:
2936:
2930:
2929:
2904:(4307): 943–60.
2893:
2887:
2886:
2868:
2836:
2830:
2829:
2819:
2787:
2781:
2772:
2766:
2765:
2763:
2762:
2735:
2729:
2728:
2726:
2725:
2714:
2708:
2707:
2705:
2704:
2689:
2683:
2682:
2672:
2654:
2645:
2639:
2638:
2628:
2610:
2601:
2595:
2594:
2592:
2591:
2577:
2571:
2570:
2568:
2567:
2553:
2547:
2546:
2544:
2543:
2528:
2522:
2521:
2519:
2518:
2503:
2497:
2496:
2494:
2493:
2478:
2472:
2471:
2469:
2468:
2462:
2454:
2448:
2447:
2445:
2444:
2438:
2427:
2419:
2413:
2412:
2402:
2378:
2372:
2371:
2361:
2351:
2319:
2313:
2312:
2268:
2262:
2261:
2233:
2227:
2226:
2216:
2184:
2178:
2177:
2167:
2158:(49): 35878–86.
2143:
2137:
2136:
2117:10.1038/370295a0
2088:
2082:
2081:
2044:
2038:
2037:
2017:
2011:
2010:
1990:
1984:
1983:
1947:
1941:
1940:
1938:
1937:
1928:. Archived from
1922:
1916:
1915:
1913:
1911:
1903:
1877:
1867:
1857:
1832:
1826:
1825:
1789:
1783:
1782:
1754:
1748:
1747:
1719:
1713:
1712:
1675:
1669:
1666:
1660:
1659:
1653:
1645:
1643:
1642:
1631:
1625:
1624:
1622:
1620:
1606:
1600:
1599:
1588:
1582:
1581:
1561:
1555:
1554:
1517:
1511:
1510:
1491:10.1038/383685a0
1466:
1460:
1459:
1445:
1439:
1438:
1420:
1414:
1413:
1377:
1371:
1370:
1360:
1351:(9): 1235–1242.
1329:
1323:
1322:
1320:
1318:
1304:
1136:Viral hypothesis
1050:
1043:
1039:
1036:
1030:
999:
991:
944:encephalopathies
927:Papua New Guinea
899:pituitary glands
481:
477:
406:genome (such as
269:Invariably fatal
133:
132:
88:
68:
67:
58:
55:
49:
27:
26:
19:
3866:
3865:
3861:
3860:
3859:
3857:
3856:
3855:
3841:
3840:
3839:
3834:
3833:
3810:
3809:
3734:
3720:
3715:
3684:
3670:Popular culture
3658:
3637:
3594:
3514:
3509:
3479:
3474:
3432:
3422:
3400:
3394:
3368:
3325:
3319:
3310:
3269:
3246:
3245:
3232:
3231:
3227:
3211:
3210:
3155:
3151:
3142:
3140:
3126:Clin Invest Med
3118:
3114:
3098:
3097:
3071:(9006): 921–5.
3060:
3056:
3040:
3039:
3005:(6197): 390–2.
2994:
2990:
2974:
2973:
2947:(9204): 51–61.
2937:
2933:
2894:
2890:
2837:
2833:
2788:
2784:
2773:
2769:
2760:
2758:
2744:Clin Invest Med
2736:
2732:
2723:
2721:
2716:
2715:
2711:
2702:
2700:
2691:
2690:
2686:
2652:
2646:
2642:
2608:
2602:
2598:
2589:
2587:
2579:
2578:
2574:
2565:
2563:
2555:
2554:
2550:
2541:
2539:
2530:
2529:
2525:
2516:
2514:
2504:
2500:
2491:
2489:
2480:
2479:
2475:
2466:
2464:
2460:
2456:
2455:
2451:
2442:
2440:
2436:
2425:
2421:
2420:
2416:
2379:
2375:
2320:
2316:
2269:
2265:
2234:
2230:
2185:
2181:
2144:
2140:
2089:
2085:
2045:
2041:
2018:
2014:
1991:
1987:
1948:
1944:
1935:
1933:
1924:
1923:
1919:
1905:
1897:
1895:
1878:
1848:(1): e1001257.
1833:
1829:
1794:J Clin Neurosci
1790:
1786:
1755:
1751:
1720:
1716:
1676:
1672:
1667:
1663:
1647:
1646:
1640:
1638:
1633:
1632:
1628:
1618:
1616:
1608:
1607:
1603:
1590:
1589:
1585:
1562:
1558:
1518:
1514:
1467:
1463:
1447:
1446:
1442:
1422:
1421:
1417:
1378:
1374:
1330:
1326:
1316:
1314:
1306:
1305:
1301:
1296:
1284:
1222:
1193:
1185:supportive care
1181:
1163:
1138:
1129:
1123:
1094:
1051:
1040:
1034:
1031:
1016:
1000:
989:
984:
950:(caused by the
901:, from cadaver
833:
709:Human diseases
612:
473:
298:and affect the
259:Palliative care
127:
99:cerebral cortex
66:
59:
53:
50:
44:
28:
24:
17:
12:
11:
5:
3864:
3854:
3853:
3836:
3835:
3832:
3831:
3819:
3818:
3816:
3812:
3811:
3808:
3807:
3796:
3785:
3770:
3755:
3735:
3730:
3729:
3727:
3726:Classification
3717:
3716:
3714:
3713:
3708:
3703:
3701:Prion diseases
3698:
3692:
3690:
3686:
3685:
3683:
3682:
3677:
3672:
3666:
3664:
3660:
3659:
3657:
3656:
3651:
3645:
3643:
3639:
3638:
3636:
3635:
3630:
3625:
3620:
3615:
3610:
3604:
3602:
3596:
3595:
3593:
3592:
3587:
3586:
3585:
3575:
3574:
3573:
3563:
3558:
3553:
3548:
3543:
3538:
3533:
3528:
3522:
3520:
3516:
3515:
3508:
3507:
3500:
3493:
3485:
3476:
3475:
3473:
3472:
3467:
3462:
3457:
3452:
3447:
3442:
3436:
3434:
3431:Prion diseases
3428:
3427:
3424:
3423:
3421:
3420:
3415:
3410:
3404:
3402:
3401:transmissible:
3396:
3395:
3393:
3392:
3387:
3382:
3376:
3374:
3370:
3369:
3367:
3366:
3361:
3356:
3351:
3346:
3340:
3338:
3327:
3324:Prion diseases
3321:
3320:
3313:Prion diseases
3309:
3308:
3301:
3294:
3286:
3280:
3279:
3268:
3267:External links
3265:
3264:
3263:
3244:
3243:
3238:Manuelidis Lab
3225:
3172:(6): 1965–70.
3149:
3112:
3054:
2988:
2931:
2888:
2831:
2782:
2767:
2730:
2709:
2684:
2640:
2596:
2572:
2548:
2523:
2498:
2473:
2449:
2414:
2373:
2334:(23): 9741–6.
2314:
2263:
2228:
2199:(7): 3453–61.
2179:
2138:
2083:
2056:(3): 660–667.
2039:
2012:
1985:
1958:(8): 1765–73.
1942:
1917:
1842:PLOS Pathogens
1827:
1784:
1749:
1714:
1670:
1661:
1626:
1601:
1583:
1572:(2): 102–110.
1556:
1529:(8): 1075–81.
1512:
1461:
1440:
1415:
1372:
1324:
1298:
1297:
1295:
1292:
1291:
1290:
1283:
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1221:
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1134:
1122:
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1117:
1116:
1115:
1112:
1109:
1106:
1103:
1093:
1090:
1053:
1052:
1003:
1001:
994:
988:
985:
983:
980:
964:Krabbe disease
895:growth hormone
832:
829:
826:
825:
823:
821:
819:
816:
813:
812:
809:
806:
803:
797:
793:
792:
789:
786:
783:
777:
773:
772:
770:
767:
764:
763:(vCJD, nvCJD)
758:
755:
754:
751:
748:
745:
739:
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734:
731:
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569:
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532:Scrapie prion
530:
521:
516:
512:
511:
504:
503:
500:
494:
491:
488:
485:
472:
471:Classification
469:
304:nervous system
292:prion diseases
279:
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261:
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22:
15:
9:
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3:
2:
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3790:
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3780:
3779:
3775:
3771:
3769:
3765:
3764:
3760:
3756:
3754:
3750:
3746:
3745:
3741:
3737:
3736:
3733:
3728:
3724:
3712:
3709:
3707:
3704:
3702:
3699:
3697:
3694:
3693:
3691:
3687:
3681:
3678:
3676:
3673:
3671:
3668:
3667:
3665:
3661:
3655:
3652:
3650:
3647:
3646:
3644:
3640:
3634:
3631:
3629:
3626:
3624:
3621:
3619:
3616:
3614:
3611:
3609:
3606:
3605:
3603:
3601:
3597:
3591:
3588:
3584:
3581:
3580:
3579:
3576:
3572:
3569:
3568:
3567:
3566:Placentophagy
3564:
3562:
3559:
3557:
3554:
3552:
3549:
3547:
3544:
3542:
3539:
3537:
3534:
3532:
3529:
3527:
3526:Autovampirism
3524:
3523:
3521:
3517:
3513:
3506:
3501:
3499:
3494:
3492:
3487:
3486:
3483:
3471:
3468:
3466:
3463:
3461:
3458:
3456:
3453:
3451:
3448:
3446:
3443:
3441:
3438:
3437:
3435:
3429:
3419:
3416:
3414:
3411:
3409:
3406:
3405:
3403:
3397:
3391:
3388:
3386:
3383:
3381:
3378:
3377:
3375:
3371:
3365:
3362:
3360:
3357:
3355:
3352:
3350:
3347:
3345:
3342:
3341:
3339:
3336:
3331:
3328:
3322:
3318:
3314:
3307:
3302:
3300:
3295:
3293:
3288:
3287:
3284:
3278:
3274:
3271:
3270:
3262:
3261:
3259:
3255:
3253:
3248:
3247:
3239:
3235:
3229:
3221:
3215:
3207:
3203:
3198:
3193:
3188:
3183:
3179:
3175:
3171:
3167:
3163:
3159:
3153:
3139:
3135:
3131:
3127:
3123:
3116:
3108:
3102:
3094:
3090:
3086:
3082:
3078:
3074:
3070:
3066:
3058:
3050:
3044:
3036:
3032:
3028:
3024:
3020:
3016:
3012:
3008:
3004:
3000:
2992:
2984:
2978:
2970:
2966:
2962:
2958:
2954:
2950:
2946:
2942:
2935:
2927:
2923:
2919:
2915:
2911:
2907:
2903:
2899:
2892:
2884:
2880:
2876:
2872:
2867:
2866:10.1038/29862
2862:
2858:
2854:
2850:
2846:
2842:
2835:
2827:
2823:
2818:
2813:
2809:
2805:
2801:
2797:
2793:
2786:
2780:
2776:
2771:
2757:
2753:
2749:
2745:
2741:
2734:
2720:. www.cdc.gov
2719:
2713:
2698:
2694:
2688:
2680:
2676:
2671:
2666:
2662:
2658:
2651:
2644:
2636:
2632:
2627:
2622:
2618:
2614:
2607:
2600:
2586:
2582:
2576:
2562:
2558:
2552:
2537:
2533:
2527:
2513:
2509:
2502:
2487:
2483:
2477:
2459:
2453:
2439:on 2012-03-31
2435:
2431:
2424:
2418:
2410:
2406:
2401:
2396:
2392:
2388:
2384:
2377:
2369:
2365:
2360:
2355:
2350:
2345:
2341:
2337:
2333:
2329:
2325:
2318:
2310:
2306:
2302:
2298:
2294:
2290:
2286:
2282:
2278:
2274:
2267:
2259:
2255:
2251:
2247:
2243:
2239:
2232:
2224:
2220:
2215:
2210:
2206:
2202:
2198:
2194:
2190:
2183:
2175:
2171:
2166:
2161:
2157:
2153:
2149:
2142:
2134:
2130:
2126:
2122:
2118:
2114:
2110:
2106:
2102:
2098:
2094:
2087:
2079:
2075:
2071:
2067:
2063:
2059:
2055:
2051:
2043:
2035:
2031:
2028:(2): 177–82.
2027:
2024:(in French).
2023:
2016:
2008:
2004:
2001:(9): 758–60.
2000:
1996:
1989:
1981:
1977:
1973:
1969:
1965:
1961:
1957:
1953:
1946:
1931:
1927:
1921:
1909:
1901:
1894:
1890:
1886:
1882:
1875:
1871:
1866:
1861:
1856:
1851:
1847:
1843:
1839:
1831:
1823:
1819:
1815:
1811:
1807:
1803:
1800:(5): 387–97.
1799:
1795:
1788:
1780:
1776:
1772:
1768:
1764:
1760:
1753:
1745:
1741:
1737:
1733:
1730:(3): 277–98.
1729:
1725:
1718:
1710:
1706:
1702:
1698:
1694:
1690:
1687:(2): 138–46.
1686:
1682:
1674:
1665:
1657:
1651:
1636:
1630:
1615:
1611:
1605:
1597:
1593:
1587:
1579:
1575:
1571:
1567:
1560:
1552:
1548:
1544:
1540:
1536:
1532:
1528:
1524:
1516:
1508:
1504:
1500:
1496:
1492:
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1484:
1480:
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1472:
1465:
1457:
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1444:
1436:
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1419:
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1403:
1399:
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1391:
1387:
1383:
1376:
1368:
1364:
1359:
1354:
1350:
1346:
1342:
1340:
1334:
1328:
1313:
1309:
1303:
1299:
1289:
1288:Proteinopathy
1286:
1285:
1279:
1277:
1273:
1269:
1264:
1260:
1256:
1252:
1248:
1244:
1239:
1235:
1231:
1227:
1217:
1213:
1209:
1205:
1203:
1197:
1188:
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1176:
1173:
1167:
1153:
1150:
1146:
1145:
1144:
1143:
1142:
1133:
1128:
1113:
1110:
1107:
1104:
1101:
1100:
1099:
1098:
1097:
1089:
1085:
1081:
1078:
1075:
1071:
1070:prion protein
1066:
1063:
1060:
1049:
1046:
1038:
1028:
1024:
1020:
1014:
1013:
1009:
1004:This section
1002:
998:
993:
992:
979:
977:
973:
969:
965:
961:
957:
953:
949:
945:
940:
937:
935:
932:
928:
924:
920:
916:
912:
908:
907:brain surgery
904:
900:
896:
891:
887:
885:
881:
877:
873:
869:
864:
862:
858:
854:
850:
846:
842:
838:
824:
822:
820:
817:
815:
814:
810:
807:
804:
801:
798:
795:
794:
790:
787:
784:
781:
778:
775:
774:
771:
768:
765:
762:
759:
757:
756:
752:
749:
746:
743:
740:
737:
736:
732:
729:
726:
724:
719:
716:
713:
712:
707:
703:
701:
698:
696:
693:
690:
687:
685:
684:
680:
677:
674:
672:
668:
665:
662:
659:
656:
655:
651:
648:
645:
643:
640:
637:
634:
631:
630:
626:
623:
620:
618:
615:
610:
607:
604:
603:
599:
596:
593:
591:
587:
583:
579:
576:
573:
570:
567:
566:
562:
559:
556:
554:
551:
548:
545:
542:
541:
537:
534:
531:
529:
525:
522:
520:
517:
514:
513:
510:
505:
501:
499:
495:
492:
490:Natural host
489:
487:Disease name
486:
483:
482:
476:
468:
465:
464:sterilization
459:
457:
453:
449:
445:
441:
437:
433:
429:
425:
421:
417:
413:
409:
405:
401:
396:
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382:
378:
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370:
366:
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349:
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321:
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309:
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297:
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285:
276:
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268:
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262:
258:
254:
250:
246:
242:
240:
236:
232:
230:
226:
223:
220:
216:
213:
209:
205:
201:
197:
193:
189:
185:
181:
177:
173:
169:
165:
161:
158:
154:
150:
146:
142:
140:
136:
131:
125:
122:
120:
116:
112:
108:
107:H&E stain
104:
100:
96:
91:
87:
82:
79:Prion disease
78:
74:
69:
57:
47:
43:
41:
35:
32:This article
30:
21:
20:
3822:
3798:
3787:
3772:
3757:
3738:
3706:Vorarephilia
3700:
3613:The Americas
3316:
3249:
3237:
3228:
3214:cite journal
3169:
3165:
3158:Manuelidis L
3152:
3141:. Retrieved
3132:(3): 101–4.
3129:
3125:
3115:
3101:cite journal
3068:
3064:
3057:
3043:cite journal
3002:
2998:
2991:
2977:cite journal
2944:
2940:
2934:
2901:
2897:
2891:
2851:(6680): 11.
2848:
2844:
2834:
2799:
2795:
2785:
2770:
2759:. Retrieved
2750:(3): 101–4.
2747:
2743:
2733:
2722:. Retrieved
2712:
2701:. Retrieved
2697:the original
2687:
2660:
2656:
2643:
2616:
2612:
2599:
2588:. Retrieved
2584:
2575:
2564:. Retrieved
2560:
2551:
2540:. Retrieved
2536:www.anses.fr
2535:
2526:
2515:. Retrieved
2511:
2501:
2490:. Retrieved
2488:. 2019-10-21
2485:
2476:
2465:. Retrieved
2452:
2441:. Retrieved
2434:the original
2429:
2417:
2390:
2386:
2376:
2331:
2327:
2317:
2276:
2272:
2266:
2244:(3): 660–7.
2241:
2237:
2231:
2196:
2192:
2182:
2155:
2151:
2141:
2100:
2096:
2086:
2053:
2049:
2042:
2025:
2021:
2015:
1998:
1994:
1988:
1955:
1951:
1945:
1934:. Retrieved
1930:the original
1920:
1906:{{
1898:{{
1845:
1841:
1830:
1797:
1793:
1787:
1762:
1758:
1752:
1727:
1723:
1717:
1684:
1680:
1673:
1664:
1639:. Retrieved
1629:
1617:. Retrieved
1613:
1604:
1586:
1569:
1565:
1559:
1526:
1522:
1515:
1474:
1470:
1464:
1452:
1443:
1435:the original
1427:
1418:
1385:
1381:
1375:
1348:
1344:
1338:
1327:
1315:. Retrieved
1311:
1302:
1223:
1214:
1210:
1206:
1202:transmission
1198:
1194:
1191:Epidemiology
1182:
1168:
1164:
1139:
1130:
1095:
1086:
1082:
1067:
1056:
1041:
1032:
1017:Please help
1005:
941:
938:
892:
888:
865:
857:histological
841:astrocytosis
834:
671:greater kudu
484:ICTVdb Code
474:
460:
444:conformation
397:
374:
350:
327:
310:, including
291:
287:
283:
282:
229:Risk factors
54:January 2022
51:
37:
33:
3512:Cannibalism
2663:: 198–201.
2619:: 206–207.
2585:www.cdc.gov
2561:www.cdc.gov
1926:"hafci.org"
1388:(1): 1–20.
1339:Spiroplasma
1230:Hippocrates
915:cannibalism
766:vCJD prion
727:Kuru prion
493:Prion name
329:Spiroplasma
148:Usual onset
76:Other names
3800:DiseasesDB
3680:Literature
3663:In fiction
3642:In animals
3333:inherited/
3143:2011-06-20
2761:2011-06-20
2724:2017-11-09
2703:2017-11-09
2590:2017-11-09
2566:2017-11-09
2542:2017-11-09
2517:2024-09-20
2492:2024-07-17
2467:2011-08-19
2443:2011-08-21
2393:: 99–108.
1936:2007-12-02
1765:: 519–50.
1641:2019-03-13
1333:Bastian FO
1294:References
1149:lentivirus
1125:See also:
1035:April 2018
925:people of
868:depression
845:astrocytes
805:FFI prion
785:GSS prion
747:CJD prion
675:EUE prion
646:FSE prion
621:BSE prion
594:CWD prion
557:TME prion
507:Non-human
432:iatrogenic
379:in sheep,
342:microscope
248:Prevention
113:(0.03 mm).
3829:neuro/662
3824:eMedicine
3696:Man-eater
3600:In humans
3399:acquired/
3373:sporadic:
3326:in humans
2883:205000018
2125:1476-4687
2070:0006-291X
1952:Neurology
1908:retracted
1900:retracted
1523:Neurology
1402:1471-8391
1253:in 1921.
1179:Treatment
1161:Diagnosis
1077:mutations
1006:does not
911:autoclave
876:myoclonus
586:mule deer
502:Ruminant
428:aetiology
274:Frequency
265:Prognosis
256:Treatment
119:Specialty
3845:Category
3256:and the
3206:17267596
3138:16021982
3093:14230097
2969:23212525
2961:14723996
2756:16021982
2679:15760286
2635:15760286
2409:14522852
2368:17535913
2309:13366031
2301:14593181
2258:12914801
2223:11238871
2174:17923484
2078:12914801
2034:11965173
1972:10563626
1893:39024193
1874:21249178
1822:31976428
1814:11535002
1779:11283320
1709:22600579
1650:cite web
1619:23 April
1578:16823692
1551:25292433
1543:11071481
1410:14522845
1367:17761489
1317:23 April
1282:See also
987:Genetics
952:JC virus
934:ruminant
931:rendered
884:dementia
880:insomnia
590:red deer
412:bacteria
395:prions.
306:of many
139:Symptoms
95:vacuoles
3794:D017096
3649:Poultry
3628:Oceania
3561:Oophagy
3556:Medical
3519:By type
3450:Scrapie
3197:1794316
3174:Bibcode
3085:8598754
3035:4351199
3027:2904126
3007:Bibcode
2906:Bibcode
2898:Science
2875:9590681
2853:Bibcode
2826:9857129
2817:1114482
2359:1887554
2336:Bibcode
2281:Bibcode
2273:Science
2133:8035877
2105:Bibcode
2007:9308333
1980:2915734
1865:3020930
1744:7788281
1701:9266722
1507:4355186
1499:8878476
1479:Bibcode
1247:scrapie
1243:scrapie
1238:scrapie
1220:History
1027:removed
1012:sources
956:CADASIL
853:primate
849:amyloid
837:neurons
519:Scrapie
509:mammals
498:isoform
420:protein
377:scrapie
338:autopsy
308:animals
180:scrapie
111:microns
42:weight.
3654:Spider
3623:Europe
3608:Africa
3590:Sexual
3546:Filial
3277:Curlie
3204:
3194:
3136:
3091:
3083:
3065:Lancet
3033:
3025:
2999:Nature
2967:
2959:
2941:Lancet
2926:142303
2924:
2881:
2873:
2845:Nature
2824:
2814:
2754:
2677:
2633:
2407:
2366:
2356:
2307:
2299:
2256:
2221:
2214:114138
2211:
2172:
2131:
2123:
2097:Nature
2076:
2068:
2032:
2005:
1978:
1970:
1891:
1887:,
1872:
1862:
1820:
1812:
1777:
1742:
1724:Micron
1707:
1699:
1576:
1549:
1541:
1505:
1497:
1471:Nature
1408:
1400:
1365:
976:heroin
968:enzyme
960:NOTCH3
872:ataxia
802:(FFI)
782:(GSS)
744:(CJD)
723:Humans
691:(CSE)
663:(EUE)
638:(FSE)
617:Cattle
574:(CWD)
549:(TME)
448:cattle
440:allele
438:prion
436:mutant
363:, and
334:cortex
324:prions
318:, and
316:cattle
312:humans
296:prions
251:Varies
218:Causes
126:
3805:25165
3571:human
3536:Endo-
3531:Child
3089:S2CID
3031:S2CID
2965:S2CID
2879:S2CID
2653:(PDF)
2609:(PDF)
2461:(PDF)
2437:(PDF)
2426:(PDF)
2305:S2CID
1976:S2CID
1904:with
1818:S2CID
1705:S2CID
1547:S2CID
1503:S2CID
1276:prion
1259:prion
1074:Point
982:Cause
695:Camel
667:Nyala
611:(BSE)
528:goats
524:Sheep
424:brain
416:prion
408:virus
320:sheep
300:brain
222:Prion
156:Types
40:undue
3789:MeSH
3778:9-CM
3753:8E0Z
3749:8E00
3675:Film
3633:List
3618:Asia
3583:list
3578:Self
3541:Exo-
3418:Kuru
3413:vCJD
3408:iCJD
3380:sCJD
3344:fCJD
3335:PRNP
3315:and
3220:link
3202:PMID
3134:PMID
3107:link
3081:PMID
3049:link
3023:PMID
2983:link
2957:PMID
2922:PMID
2871:PMID
2822:PMID
2752:PMID
2675:PMID
2631:PMID
2405:PMID
2364:PMID
2297:PMID
2254:PMID
2219:PMID
2170:PMID
2129:PMID
2121:ISSN
2074:PMID
2066:ISSN
2030:PMID
2003:PMID
1968:PMID
1889:PMID
1870:PMID
1810:PMID
1775:PMID
1740:PMID
1697:PMID
1656:link
1621:2023
1574:PMID
1539:PMID
1495:PMID
1406:PMID
1398:ISSN
1363:PMID
1319:2023
1062:gene
1059:PRNP
1010:any
1008:cite
923:Fore
919:kuru
903:dura
808:PrP
788:PrP
769:PrP
750:PrP
730:PrP
718:Kuru
704:Yes
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