1182:
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.
97:
141:
1211:(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.
1173:
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.
1215:
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.
1219:
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.
1004:
36:
1264:'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
1202:
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
1214:
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
900:
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
1222:
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
1172:
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
1218:
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
1071:
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
1181:
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
1138:
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
1206:
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
1247:
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.
3003:
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".
2054:
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".
382:
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.
3069:
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".
1090:
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.
1223:
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.
1685:
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".
1527:
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".
1842:
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).
477:
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.
472:
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
1147:
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:
1641:
1272:
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
1086:
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.
1094:
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.
3797:
3782:
3763:
1176:
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
1121:
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
1799:
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".
1203:
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.
985:
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.
862:
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
1072:
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.
1079:(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.
2243:
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".
846:
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
924:" 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
2539:"Transmissible Spongiform Encephalopathies (TSEs), also known as prion diseases | Anses - Agence nationale de sécurité sanitaire de l'alimentation, de l'environnement et du travail"
3290:
465:, 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
904:
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
1268:
protein might transmit spongiform encephalopathy. Not until 1988 was the neuropathology of spongiform encephalopathy properly described in cows. The alarming amplification of
2781:
3068:
3734:
1103:
Protein could be the infectious agent, inducing its own replication by causing conformational change of normal cellular PrP into PrP. Evidence for this hypothesis:
457:. Transmission occurs when healthy animals consume tainted tissues from others with the disease. In the 1980s and 1990s, bovine spongiform encephalopathy spread in
1476:
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".
1025:
445:
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
1285:
transplantation model that explains why cannibalism favours transmission, the search for a viral agent was, as of 2007, being continued in some laboratories.
870:
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
1957:
Kriegstein AR; Shungu DC; Millar WS; et al. (1999). "Leukoencephalopathy and raised brain lactate from heroin vapor inhalation ("chasing the dragon")".
3002:
3874:
3372:
2278:
Mallucci G; Dickinson A; Lineham J; et al. (October 2003). "Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis".
1190:
There are currently no known ways to cure or prevent prion disease. Certain medications can slow down the progression of the disease. But ultimately,
367:
3226:
3113:
3055:
2989:
1729:
Jeffrey M, Goodbrand IA, Goodsir CM (1995). "Pathology of the transmissible spongiform encephalopathies with special emphasis on ultrastructure".
2098:
Collinge, John; Whittington, Miles A.; Sidle, Katie C. L.; Smith, Corinne J.; Palmer, Mark S.; Clarke, Anthony R.; Jefferys, John G. R. (1994).
1252:
between animals, until extraordinary measures were taken such as the intra-ocular injection of infected nervous tissue. No direct link between
244:
Contact with infected fluids, ingestion of infected flesh, having one or two parents that have the disease (in case of fatal familial insomnia)
2538:
1571:
Colle, JG; Bradley, R; Libersky, PP (2006). "Variant CJD (vCJD) and bovine spongiform encephalopathy (BSE): 10 and 20 years on: part 2".
901:
of spongiform change as a diagnostic feature, reflected in the use of the term "spongiform encephalopathy" for this group of disorders.
1460:
1178:
958:
790:
202:
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2429:
1314:
3413:
2699:
1430:
1133:
379:
194:
134:
981:(PSL)—which is a spongiform encephalopathy—is also probably not caused by a prion, although the adulterant that causes it among
2746:
3526:
3436:
3387:
771:
2155:"High titers of transmissible spongiform encephalopathy infectivity associated with extremely low levels of PrPSc in vivo"
1932:
3257:
This entry incorporates public domain text originally from the National Institute of Neurological Disorders and Stroke,
3128:
1617:"ICTVdB : the universal virus database of the International Committee on Taxonomy of Viruses - NLM Catalog - NCBI"
3606:
3483:
3264:
1051:
557:
466:
399:
210:
1033:
3656:
3488:
3463:
3169:"Cells infected with scrapie and Creutzfeldt–Jakob disease agents produce intracellular 25-nm virus-like particles"
2785:
1278:
1269:
1240:
646:
619:
403:
391:
206:
170:
3468:
1387:
Chesebro, Bruce (2003-06-01). "Introduction to the transmissible spongiform encephalopathies or prion diseases".
1243:
records cases of a disease with similar characteristics in the 4th and 5th centuries AD. In 1755, an outbreak of
699:
218:
1348:
spp. from transmissible spongiform encephalopathy brains or ticks induce spongiform encephalopathy in ruminants"
866:
in 1966, followed by CJD in 1968 and GSS in 1981. These neuropathological features have formed the basis of the
3693:
3493:
1029:
947:
proteins in ruminant feed as a precaution against the spread of prion infection in cattle and other ruminants.
671:
214:
56:
3431:
3403:
3367:
3305:
3258:
2946:
Collins SJ, Lawson VA, Masters CL.; Lawson; Masters (Jan 2004). "Transmissible spongiform encephalopathies".
2488:
1662:
1274:
893:, confusion, or memory problems. In the later stages of the disease, patients have severe mental impairment (
752:
363:
355:. The disorders cause impairment of brain function which may result in memory loss, personality changes, and
113:
17:
3776:
3320:
474:
2489:"Therapeutic Approaches for Prion Diseases | NIAID: National Institute of Allergy and Infectious Diseases"
877:
The clinical signs in humans vary, but commonly include personality changes, psychiatric problems such as
3636:
433:
material. Misfolded prion proteins carry the disease between individuals and cause deterioration of the
3163:
96:
940:, who used to consume their dead as a funerary ritual. Laws in developed countries now ban the use of
3703:
3519:
1435:
978:
178:
3812:
3382:
3129:"Sacred disease of our times: failure of the infectious disease model of spongiform encephalopathy"
2747:"Sacred disease of our times: failure of the infectious disease model of spongiform encephalopathy"
1261:
1256:
and human disease was suspected then or has been found since. TSE was first described in humans by
1014:
928:
or similar practices allow the proteins to accumulate over more than one generation. An example is
222:
140:
3478:
3313:
1602:
1208:
1018:
810:
582:
395:
371:
198:
174:
3261:
2676:
2632:
2588:"Surveillance for vCJD | Variant Creutzfeldt-Jakob Disease, Classic (CJD) | Prion Disease | CDC"
3672:
3651:
2464:
941:
871:
3772:
2514:
3646:
3631:
3220:
3107:
3049:
2983:
1764:
Collinge J (2001). "Prion diseases of humans and animals: their causes and molecular basis".
1455:
1239:
described a disease like TSE in cattle and sheep, which he believed also occurred in humans.
1191:
3512:
3180:
3013:
2912:
2903:
Gajdusek DC (Sep 1977). "Unconventional viruses and the origin and disappearance of kuru".
2859:
2656:
2612:
2342:
2287:
2111:
2000:
Chang YJ, Tsai CH, Chen CJ (1997). "Leukoencephalopathy after inhalation of heroin vapor".
1485:
8:
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3641:
3594:
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438:
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3017:
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2115:
1489:
3677:
3613:
3569:
3203:
3168:
3095:
3037:
2971:
2885:
2823:
2798:
2725:"Risk for Travelers | Variant Creutzfeldt-Jakob Disease, Classic (CJD) | Prion Disease"
2703:
2365:
2330:
2311:
1982:
1914:
1906:
1871:
1844:
1824:
1711:
1616:
1553:
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infection. Mental and physical abilities deteriorate and many tiny holes appear in the
305:, are a group of progressive, incurable, and fatal conditions that are associated with
149:
3240:
3083:
2959:
2724:
2256:
2220:
2195:
2068:
950:
There exists evidence that prion diseases may be transmissible by the airborne route.
486:
Differences in shape between the different prion protein forms are poorly understood.
3823:
3623:
3554:
3208:
3140:
3087:
3029:
2963:
2928:
2889:
2877:
2828:
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2681:
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2411:
2370:
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2135:
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2080:
2072:
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2009:
1974:
1895:
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1816:
1781:
1746:
1742:
1703:
1656:
1580:
1545:
1501:
1412:
1404:
1369:
1342:, Sanders DE, Forbes WA, Hagius SD, Walker JV, Henk WG, Enright FM, Elzer PH (2007).
925:
592:
454:
453:, which expresses prion proteins that contort by themselves into the disease-causing
441:
may be genetic, sporadic, or infectious via ingestion of infected foodstuffs and via
249:
129:
3852:
3806:
3099:
2975:
2587:
2564:"Infection Control | Creutzfeldt-Jakob Disease, Classic (CJD) | Prion Disease | CDC"
2315:
2211:
1828:
1715:
1557:
3601:
3198:
3188:
3167:, Yu ZX, Barquero N, Banquero N, Mullins B; Yu; Banquero; Mullins (February 2007).
3164:
3079:
3041:
3021:
2955:
2920:
2867:
2818:
2810:
2799:"1755 and all that: a historical primer of transmissible spongiform encephalopathy"
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2401:
2360:
2350:
2295:
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2215:
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2119:
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1986:
1966:
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1808:
1777:
1773:
1738:
1695:
1537:
1513:
1493:
1396:
1359:
1339:
1139:
prion, or they may serve as catalysts for the replication of a protein-only prion.
937:
1598:
1281:. Although the infectious disease model of TSE has been questioned in favour of a
3817:
3559:
3285:
2465:"SOFIA: An Assay Platform for Ultrasensitive Detection of PrP in Brain and Blood"
1891:
1861:
909:
344:
109:
50:
3267:
1456:"Variant Creutzfeldt-Jakob disease > Relationship with BSE (Mad Cow Disease)"
3828:
3564:
3441:
3408:
3377:
3173:
Proceedings of the National Academy of Sciences of the United States of America
2945:
2335:
Proceedings of the National Academy of Sciences of the United States of America
1936:
1080:
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954:
929:
905:
728:
375:
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182:
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2814:
3868:
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2131:
2076:
1408:
1294:
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933:
917:
3791:
3193:
2355:
2299:
1642:"Deux chercheurs algériens découvrent la maladie du "chameau fou" à Ouargla"
409:
Unlike other kinds of infectious disease, which are spread by agents with a
3729:
3212:
3144:
2967:
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2762:
2685:
2641:
2415:
2374:
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2229:
2180:
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2099:
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2040:
1978:
1899:
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1820:
1812:
1785:
1584:
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1416:
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851:
681:
442:
117:
3091:
3033:
2881:
2832:
2406:
2389:
2139:
2013:
1970:
1903:. If this is an intentional citation to a retracted paper, please replace
1750:
1707:
1699:
1541:
1505:
1364:
1343:
3535:
2932:
1400:
1236:
339:
254:
Currently there is no way to reliably detect prions except at post-mortem
239:
1109:
Infectivity titre correlates with PrP levels. However, this is disputed.
1155:
913:
855:
394:(BSE) in cattle – popularly known as "mad cow disease" – and
352:
333:. According to the most widespread hypothesis, they are transmitted by
3755:
2277:
1845:"Aerosols Transmit Prions to Immunocompetent and Immunodeficient Mice"
3847:
3719:
3025:
2123:
2027:
Koussa S, Zabad R, Rizk T, Tamraz J, Nasnas R, Chemaly R (2002). "".
1497:
1075:
The PRNP gene provides the instructions to make a protein called the
921:
886:
885:). Patients also may experience involuntary jerking movements called
867:
596:
275:
154:
Dementia, seizures, tremors, insomnia, psychosis, delirium, confusion
1684:
1003:
932:, which reached epidemic proportions in the mid-20th century in the
1083:
962:
944:
894:
890:
600:
446:
422:
2872:
2847:
1841:
1599:"Prion Disinfection Options - Biosafety & Occupational Health"
461:
in an epidemic fashion. This occurred because cattle were fed the
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1253:
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causing it to appear like a sponge when brain tissue obtained at
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1956:
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121:
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1248:
Early-20th-century experiments failed to show transmission of
3336:
2331:"Formation of native prions from minimal components in vitro"
2328:
2196:"Branched Polyamines Cure Prion-Infected Neuroblastoma Cells"
2153:
Barron RM; Campbell SL; King D; et al. (December 2007).
2097:
1282:
1265:
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534:
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232:
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Deleault NR, Harris BT, Rees JR, Supattapone S (June 2007).
2194:
Supattapone S; Wille H; Uyechi L; et al. (April 2001).
2193:
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1068:
1065:
563:
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186:
2152:
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1338:
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Traditional Chinese medicines derived from the human body
2796:
2100:"Prion protein is necessary for normal synaptic function"
1232:
652:
588:
414:
410:
1728:
1319:
National Institute of Neurological Disorders and Stroke
1194:
is currently the only option for infected individuals.
2053:
1644:. 2018-05-09. Archived from the original on 2018-06-17
1526:
2654:
2610:
2242:
2026:
337:, though some other data suggest an involvement of a
3745:
1798:
1570:
425:), the infectious agent in TSEs is believed to be a
2245:
Biochemical and Biophysical Research Communications
2057:
Biochemical and Biophysical Research Communications
969:(caused by abnormal NOTCH3 protein activity), and
881:, lack of coordination, and/or an unsteady gait (
3866:
108:that appear as holes in tissue sections) in the
858:due to the destruction of nearby neurons), and
3157:
1161:Strain variation of different isolates of PrP.
3520:
3321:
2797:Brown P, Bradley R; Bradley (December 1998).
2322:
1999:
1127:
82:Transmissible spongiform encephalopathy (TSE)
3225:: CS1 maint: multiple names: authors list (
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2657:"The Public Health Impact of Prion Diseases"
2613:"The Public Health Impact of Prion Diseases"
2020:
1950:
1332:
104:Micrograph showing spongiform degeneration (
49:"Cause" section is in fairly bad shape with
2902:
2845:
2702:. World Health Organization. Archived from
2677:10.1146/annurev.publhealth.26.021304.144536
2633:10.1146/annurev.publhealth.26.021304.144536
1993:
1315:"Transmissible Spongiform Encephalopathies"
1032:. Unsourced material may be challenged and
3527:
3513:
3328:
3314:
1917:|...|intentional=yes}}
1763:
1675:Believed to be identical to the BSE prion.
1461:Centers for Disease Control and Prevention
1207:contracted the disease through iatrogenic
1179:Surround Optical Fiber Immunoassay (SOFIA)
1098:
979:Progressive Spongiform Leukoencephalopathy
139:
95:
45:needs attention from an expert in Medicine
3875:Transmissible spongiform encephalopathies
3202:
3192:
3126:
2871:
2848:"It's Jakob's disease, not Creutzfeldt's"
2822:
2744:
2675:
2631:
2405:
2364:
2354:
2219:
2170:
1870:
1860:
1363:
1052:Learn how and when to remove this message
957:are caused by prions, as in the cases of
916:allografts and from instruments used for
897:) and lose the ability to move or speak.
467:variant form of Creutzfeldt–Jakob disease
437:. TSEs are unique diseases in that their
400:variant form of Creutzfeldt–Jakob disease
295:Transmissible spongiform encephalopathies
27:Group of brain diseases induced by prions
2387:
1386:
3414:Variably protease-sensitive prionopathy
3373:Gerstmann–Sträussler–Scheinker syndrome
3341:transmissible spongiform encephalopathy
3297:Transmissible spongiform encephalopathy
1134:Protein misfolding cyclic amplification
920:(Brown, 2000) (prions can survive the "
791:Gerstmann-Sträussler-Scheinker syndrome
380:variably protease-sensitive prionopathy
368:Gerstmann–Sträussler–Scheinker syndrome
203:Gerstmann-Sträussler-Scheinker syndrome
195:variably protease-sensitive prionopathy
14:
3867:
59:may be able to help recruit an expert.
3508:
3309:
841:
378:, as well as the recently discovered
1609:
1307:
1030:adding citations to reliable sources
997:
854:(abnormal increase in the number of
624:commonly known as "mad cow disease"
29:
2700:"Variant Creutzfeldt-Jakob disease"
2512:
2159:The Journal of Biological Chemistry
1431:"Variant Creutzfeldt-Jakob disease"
1154:Incubation time is comparable to a
1142:
829:Familial spongiform encephalopathy
402:in humans is caused by exposure to
24:
2439:: 195. August 2010. Archived from
1115:Denaturing PrP removes infectivity
490:Known spongiform encephalopathies
386:TSEs in non-human mammals include
25:
3886:
3484:Transmissible mink encephalopathy
3273:
3265:U.S. National Library of Medicine
772:Variant Creutzfeldt–Jakob disease
558:Transmissible mink encephalopathy
481:
463:processed remains of other cattle
211:transmissible mink encephalopathy
3489:Feline spongiform encephalopathy
3464:Bovine spongiform encephalopathy
2786:Publius Flavius Vegetius Renatus
1241:Publius Flavius Vegetius Renatus
1118:PrP-null mice cannot be infected
1002:
647:Feline spongiform encephalopathy
620:Bovine spongiform encephalopathy
429:, thus being composed solely of
404:bovine spongiform encephalopathy
392:bovine spongiform encephalopathy
207:feline spongiform encephalopathy
171:Bovine spongiform encephalopathy
34:
3534:
3469:Camel spongiform encephalopathy
3233:
3120:
3062:
2996:
2939:
2896:
2839:
2790:
2775:
2738:
2717:
2692:
2648:
2604:
2580:
2556:
2531:
2506:
2481:
2470:. SUNY Downstate Medical Center
2457:
2422:
2381:
2271:
2236:
2212:10.1128/JVI.75.7.3453-3461.2001
2187:
2146:
2091:
2047:
1925:
1835:
1792:
1757:
1722:
1678:
1669:
1634:
1440:. February 2012. Archived from
1352:Journal of Medical Microbiology
1197:
973:(caused by a deficiency of the
700:Camel spongiform encephalopathy
219:camel spongiform encephalopathy
3494:Exotic ungulate encephalopathy
2664:Annual Review of Public Health
2655:Belay and Schonberger (2005).
2620:Annual Review of Public Health
2611:Belay and Schonberger (2005).
1778:10.1146/annurev.neuro.24.1.519
1591:
1564:
1520:
1469:
1448:
1423:
1380:
672:Exotic ungulate encephalopathy
215:exotic ungulate encephalopathy
13:
1:
3259:National Institutes of Health
3084:10.1016/S0140-6736(96)91412-9
2960:10.1016/S0140-6736(03)15171-9
2257:10.1016/S0006-291X(03)01459-1
2069:10.1016/s0006-291x(03)01459-1
1300:
357:abnormal or impaired movement
112:of a patient who had died of
1892:10.1371/journal.ppat.1012396
1862:10.1371/journal.ppat.1001257
1743:10.1016/0968-4328(95)00004-N
1185:
1167:
7:
3388:Huntington's disease-like 1
2782:Digesta Artis Mulomedicinae
2430:"Detecting Prions in Blood"
1288:
993:
398:(CWD) in deer and elk. The
187:Huntington's disease-like 1
47:. The specific problem is:
10:
3891:
3127:McAlister, V (June 2005).
2745:McAlister, V (June 2005).
1226:
1131:
1128:Multi-component hypothesis
3838:
3749:
3712:
3686:
3665:
3622:
3542:
3454:
3422:
3396:
3356:
3347:
2815:10.1136/bmj.317.7174.1688
1661:: CS1 maint: unfit URL (
1436:World Health Organization
1275:Creutzfeldt–Jakob disease
753:Creutzfeldt–Jakob disease
732:
719:
517:
364:Creutzfeldt–Jakob disease
284:
274:
266:
258:
248:
238:
228:
179:Creutzfeldt-Jakob disease
166:
158:
148:
128:
114:Creutzfeldt–Jakob disease
103:
94:
86:
81:
3383:PrP systemic amyloidosis
2846:Katscher F. (May 1998).
2394:British Medical Bulletin
1389:British Medical Bulletin
1262:Daniel Carleton Gajdusek
988:
469:in the 1990s and 2000s.
359:which worsen over time.
223:PrP systemic amyloidosis
3479:Chronic wasting disease
3409:Sporadic fatal insomnia
3378:Fatal familial insomnia
3291:considered for deletion
3194:10.1073/pnas.0610999104
2356:10.1073/pnas.0702662104
2300:10.1126/science.1090187
1603:University of Minnesota
1277:, in humans exposed to
1112:PrP is an isomer of PrP
1099:Protein-only hypothesis
977:galactosylceramidase).
811:Fatal familial insomnia
583:Chronic wasting disease
396:chronic wasting disease
372:fatal familial insomnia
362:TSEs of humans include
199:chronic wasting disease
175:Fatal familial insomnia
3241:"Infectious Particles"
2925:10.1126/science.142303
2390:"TSE strain variation"
2172:10.1074/jbc.M704329200
1813:10.1054/jocn.2001.0919
1463:. Retrieved 2017-04-25
1438:. Retrieved 2017-04-25
889:, unusual sensations,
872:central nervous system
2519:National Prion Clinic
2002:J. Formos. Med. Assoc
1971:10.1212/WNL.53.8.1765
1909:|...}}
1886:(Retracted, see
1700:10.1002/ana.410420203
1542:10.1212/WNL.55.8.1075
1444:on December 20, 2002.
1365:10.1099/jmm.0.47159-0
908:harvested from human
874:in individual cases.
2706:on December 20, 2002
2029:Rev. Neurol. (Paris)
1621:www.ncbi.nlm.nih.gov
1573:Folia Neuropatholica
1026:improve this section
351:is examined under a
57:WikiProject Medicine
3575:Kleptopharmacophagy
3185:2007PNAS..104.1965M
3018:1988Natur.336..390H
2917:1977Sci...197..943C
2864:1998Natur.393Q..11K
2437:Microbiology Today.
2407:10.1093/bmb/66.1.99
2347:2007PNAS..104.9741D
2292:2003Sci...302..871M
2200:Journal of Virology
2116:1994Natur.370..295C
1939:on November 1, 2004
1688:Annals of Neurology
1605:. 17 November 2017.
1490:1996Natur.383..685C
1465:. 10 February 2015.
1231:In the 5th century
491:
135:Infectious diseases
3839:External resources
2545:. 18 February 2013
2513:UCL (2021-02-02).
1401:10.1093/bmb/66.1.1
1258:Alfons Maria Jakob
953:Note that not all
842:Signs and symptoms
489:
3862:
3861:
3743:
3742:
3502:
3501:
3450:
3449:
2809:(7174): 1688–92.
2515:"Drug treatments"
2493:www.niaid.nih.gov
2388:Bruce ME (2003).
2286:(5646): 871–874.
2110:(6487): 295–297.
1766:Annu Rev Neurosci
1484:(6602): 685–690.
1064:Mutations in the
1062:
1061:
1054:
839:
838:
807:90.001.0.01.010.
787:90.001.0.01.009.
749:90.001.0.01.008.
725:90.001.0.01.007.
668:90.001.0.01.006.
643:90.001.0.01.005.
616:90.001.0.01.004.
593:white-tailed deer
579:90.001.0.01.003.
554:90.001.0.01.002.
526:90.001.0.01.001.
301:), also known as
292:
291:
250:Diagnostic method
162:Months to decades
120:, scale bar = 30
76:Medical condition
74:
73:
16:(Redirected from
3882:
3747:
3746:
3713:Related concepts
3529:
3522:
3515:
3506:
3505:
3457:in other animals
3354:
3353:
3330:
3323:
3316:
3307:
3306:
3294:
3249:
3248:
3237:
3231:
3230:
3224:
3216:
3206:
3196:
3161:
3155:
3154:
3152:
3151:
3124:
3118:
3117:
3111:
3103:
3066:
3060:
3059:
3053:
3045:
3026:10.1038/336390a0
3000:
2994:
2993:
2987:
2979:
2943:
2937:
2936:
2911:(4307): 943–60.
2900:
2894:
2893:
2875:
2843:
2837:
2836:
2826:
2794:
2788:
2779:
2773:
2772:
2770:
2769:
2742:
2736:
2735:
2733:
2732:
2721:
2715:
2714:
2712:
2711:
2696:
2690:
2689:
2679:
2661:
2652:
2646:
2645:
2635:
2617:
2608:
2602:
2601:
2599:
2598:
2584:
2578:
2577:
2575:
2574:
2560:
2554:
2553:
2551:
2550:
2535:
2529:
2528:
2526:
2525:
2510:
2504:
2503:
2501:
2500:
2485:
2479:
2478:
2476:
2475:
2469:
2461:
2455:
2454:
2452:
2451:
2445:
2434:
2426:
2420:
2419:
2409:
2385:
2379:
2378:
2368:
2358:
2326:
2320:
2319:
2275:
2269:
2268:
2240:
2234:
2233:
2223:
2191:
2185:
2184:
2174:
2165:(49): 35878–86.
2150:
2144:
2143:
2124:10.1038/370295a0
2095:
2089:
2088:
2051:
2045:
2044:
2024:
2018:
2017:
1997:
1991:
1990:
1954:
1948:
1947:
1945:
1944:
1935:. Archived from
1929:
1923:
1922:
1920:
1918:
1910:
1884:
1874:
1864:
1839:
1833:
1832:
1796:
1790:
1789:
1761:
1755:
1754:
1726:
1720:
1719:
1682:
1676:
1673:
1667:
1666:
1660:
1652:
1650:
1649:
1638:
1632:
1631:
1629:
1627:
1613:
1607:
1606:
1595:
1589:
1588:
1568:
1562:
1561:
1524:
1518:
1517:
1498:10.1038/383685a0
1473:
1467:
1466:
1452:
1446:
1445:
1427:
1421:
1420:
1384:
1378:
1377:
1367:
1358:(9): 1235–1242.
1336:
1330:
1329:
1327:
1325:
1311:
1143:Viral hypothesis
1057:
1050:
1046:
1043:
1037:
1006:
998:
955:encephalopathies
938:Papua New Guinea
910:pituitary glands
492:
488:
417:genome (such as
280:Invariably fatal
144:
143:
99:
79:
78:
69:
66:
60:
38:
37:
30:
21:
3890:
3889:
3885:
3884:
3883:
3881:
3880:
3879:
3865:
3864:
3863:
3858:
3857:
3834:
3833:
3758:
3744:
3739:
3708:
3694:Popular culture
3682:
3661:
3618:
3538:
3533:
3503:
3498:
3456:
3446:
3424:
3418:
3392:
3349:
3343:
3334:
3279:
3276:
3253:
3252:
3239:
3238:
3234:
3218:
3217:
3162:
3158:
3149:
3147:
3133:Clin Invest Med
3125:
3121:
3105:
3104:
3078:(9006): 921–5.
3067:
3063:
3047:
3046:
3012:(6197): 390–2.
3001:
2997:
2981:
2980:
2954:(9204): 51–61.
2944:
2940:
2901:
2897:
2844:
2840:
2795:
2791:
2780:
2776:
2767:
2765:
2751:Clin Invest Med
2743:
2739:
2730:
2728:
2723:
2722:
2718:
2709:
2707:
2698:
2697:
2693:
2659:
2653:
2649:
2615:
2609:
2605:
2596:
2594:
2586:
2585:
2581:
2572:
2570:
2562:
2561:
2557:
2548:
2546:
2537:
2536:
2532:
2523:
2521:
2511:
2507:
2498:
2496:
2487:
2486:
2482:
2473:
2471:
2467:
2463:
2462:
2458:
2449:
2447:
2443:
2432:
2428:
2427:
2423:
2386:
2382:
2327:
2323:
2276:
2272:
2241:
2237:
2192:
2188:
2151:
2147:
2096:
2092:
2052:
2048:
2025:
2021:
1998:
1994:
1955:
1951:
1942:
1940:
1931:
1930:
1926:
1912:
1904:
1902:
1885:
1855:(1): e1001257.
1840:
1836:
1801:J Clin Neurosci
1797:
1793:
1762:
1758:
1727:
1723:
1683:
1679:
1674:
1670:
1654:
1653:
1647:
1645:
1640:
1639:
1635:
1625:
1623:
1615:
1614:
1610:
1597:
1596:
1592:
1569:
1565:
1525:
1521:
1474:
1470:
1454:
1453:
1449:
1429:
1428:
1424:
1385:
1381:
1337:
1333:
1323:
1321:
1313:
1312:
1308:
1303:
1291:
1229:
1200:
1192:supportive care
1188:
1170:
1145:
1136:
1130:
1101:
1058:
1047:
1041:
1038:
1023:
1007:
996:
991:
961:(caused by the
912:, from cadaver
844:
720:Human diseases
623:
484:
309:and affect the
270:Palliative care
138:
110:cerebral cortex
77:
70:
64:
61:
55:
39:
35:
28:
23:
22:
15:
12:
11:
5:
3888:
3878:
3877:
3860:
3859:
3856:
3855:
3843:
3842:
3840:
3836:
3835:
3832:
3831:
3820:
3809:
3794:
3779:
3759:
3754:
3753:
3751:
3750:Classification
3741:
3740:
3738:
3737:
3732:
3727:
3725:Prion diseases
3722:
3716:
3714:
3710:
3709:
3707:
3706:
3701:
3696:
3690:
3688:
3684:
3683:
3681:
3680:
3675:
3669:
3667:
3663:
3662:
3660:
3659:
3654:
3649:
3644:
3639:
3634:
3628:
3626:
3620:
3619:
3617:
3616:
3611:
3610:
3609:
3599:
3598:
3597:
3587:
3582:
3577:
3572:
3567:
3562:
3557:
3552:
3546:
3544:
3540:
3539:
3532:
3531:
3524:
3517:
3509:
3500:
3499:
3497:
3496:
3491:
3486:
3481:
3476:
3471:
3466:
3460:
3458:
3455:Prion diseases
3452:
3451:
3448:
3447:
3445:
3444:
3439:
3434:
3428:
3426:
3425:transmissible:
3420:
3419:
3417:
3416:
3411:
3406:
3400:
3398:
3394:
3393:
3391:
3390:
3385:
3380:
3375:
3370:
3364:
3362:
3351:
3348:Prion diseases
3345:
3344:
3337:Prion diseases
3333:
3332:
3325:
3318:
3310:
3304:
3303:
3275:
3274:External links
3272:
3271:
3270:
3251:
3250:
3245:Manuelidis Lab
3232:
3179:(6): 1965–70.
3156:
3119:
3061:
2995:
2938:
2895:
2838:
2789:
2774:
2737:
2716:
2691:
2647:
2603:
2579:
2555:
2530:
2505:
2480:
2456:
2421:
2380:
2341:(23): 9741–6.
2321:
2270:
2235:
2206:(7): 3453–61.
2186:
2145:
2090:
2063:(3): 660–667.
2046:
2019:
1992:
1965:(8): 1765–73.
1949:
1924:
1849:PLOS Pathogens
1834:
1791:
1756:
1721:
1677:
1668:
1633:
1608:
1590:
1579:(2): 102–110.
1563:
1536:(8): 1075–81.
1519:
1468:
1447:
1422:
1379:
1331:
1305:
1304:
1302:
1299:
1298:
1297:
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1129:
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1122:
1119:
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1113:
1110:
1100:
1097:
1060:
1059:
1010:
1008:
1001:
995:
992:
990:
987:
971:Krabbe disease
906:growth hormone
843:
840:
837:
836:
834:
832:
830:
827:
824:
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820:
817:
814:
808:
804:
803:
800:
797:
794:
788:
784:
783:
781:
778:
775:
774:(vCJD, nvCJD)
769:
766:
765:
762:
759:
756:
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746:
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569:
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551:
550:
547:
544:
543:Scrapie prion
541:
532:
527:
523:
522:
515:
514:
511:
505:
502:
499:
496:
483:
482:Classification
480:
315:nervous system
303:prion diseases
290:
289:
286:
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281:
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268:
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42:
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6:
4:
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3872:
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3849:
3845:
3844:
3841:
3837:
3830:
3826:
3825:
3821:
3819:
3815:
3814:
3810:
3808:
3804:
3803:
3799:
3795:
3793:
3789:
3788:
3784:
3780:
3778:
3774:
3770:
3769:
3765:
3761:
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3757:
3752:
3748:
3736:
3733:
3731:
3728:
3726:
3723:
3721:
3718:
3717:
3715:
3711:
3705:
3702:
3700:
3697:
3695:
3692:
3691:
3689:
3685:
3679:
3676:
3674:
3671:
3670:
3668:
3664:
3658:
3655:
3653:
3650:
3648:
3645:
3643:
3640:
3638:
3635:
3633:
3630:
3629:
3627:
3625:
3621:
3615:
3612:
3608:
3605:
3604:
3603:
3600:
3596:
3593:
3592:
3591:
3590:Placentophagy
3588:
3586:
3583:
3581:
3578:
3576:
3573:
3571:
3568:
3566:
3563:
3561:
3558:
3556:
3553:
3551:
3550:Autovampirism
3548:
3547:
3545:
3541:
3537:
3530:
3525:
3523:
3518:
3516:
3511:
3510:
3507:
3495:
3492:
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3487:
3485:
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3472:
3470:
3467:
3465:
3462:
3461:
3459:
3453:
3443:
3440:
3438:
3435:
3433:
3430:
3429:
3427:
3421:
3415:
3412:
3410:
3407:
3405:
3402:
3401:
3399:
3395:
3389:
3386:
3384:
3381:
3379:
3376:
3374:
3371:
3369:
3366:
3365:
3363:
3360:
3355:
3352:
3346:
3342:
3338:
3331:
3326:
3324:
3319:
3317:
3312:
3311:
3308:
3302:
3298:
3292:
3288:
3287:
3283:
3278:
3277:
3269:
3268:
3266:
3262:
3260:
3255:
3254:
3246:
3242:
3236:
3228:
3222:
3214:
3210:
3205:
3200:
3195:
3190:
3186:
3182:
3178:
3174:
3170:
3166:
3160:
3146:
3142:
3138:
3134:
3130:
3123:
3115:
3109:
3101:
3097:
3093:
3089:
3085:
3081:
3077:
3073:
3065:
3057:
3051:
3043:
3039:
3035:
3031:
3027:
3023:
3019:
3015:
3011:
3007:
2999:
2991:
2985:
2977:
2973:
2969:
2965:
2961:
2957:
2953:
2949:
2942:
2934:
2930:
2926:
2922:
2918:
2914:
2910:
2906:
2899:
2891:
2887:
2883:
2879:
2874:
2873:10.1038/29862
2869:
2865:
2861:
2857:
2853:
2849:
2842:
2834:
2830:
2825:
2820:
2816:
2812:
2808:
2804:
2800:
2793:
2787:
2783:
2778:
2764:
2760:
2756:
2752:
2748:
2741:
2727:. www.cdc.gov
2726:
2720:
2705:
2701:
2695:
2687:
2683:
2678:
2673:
2669:
2665:
2658:
2651:
2643:
2639:
2634:
2629:
2625:
2621:
2614:
2607:
2593:
2589:
2583:
2569:
2565:
2559:
2544:
2540:
2534:
2520:
2516:
2509:
2494:
2490:
2484:
2466:
2460:
2446:on 2012-03-31
2442:
2438:
2431:
2425:
2417:
2413:
2408:
2403:
2399:
2395:
2391:
2384:
2376:
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2362:
2357:
2352:
2348:
2344:
2340:
2336:
2332:
2325:
2317:
2313:
2309:
2305:
2301:
2297:
2293:
2289:
2285:
2281:
2274:
2266:
2262:
2258:
2254:
2250:
2246:
2239:
2231:
2227:
2222:
2217:
2213:
2209:
2205:
2201:
2197:
2190:
2182:
2178:
2173:
2168:
2164:
2160:
2156:
2149:
2141:
2137:
2133:
2129:
2125:
2121:
2117:
2113:
2109:
2105:
2101:
2094:
2086:
2082:
2078:
2074:
2070:
2066:
2062:
2058:
2050:
2042:
2038:
2035:(2): 177–82.
2034:
2031:(in French).
2030:
2023:
2015:
2011:
2008:(9): 758–60.
2007:
2003:
1996:
1988:
1984:
1980:
1976:
1972:
1968:
1964:
1960:
1953:
1938:
1934:
1928:
1916:
1908:
1901:
1897:
1893:
1889:
1882:
1878:
1873:
1868:
1863:
1858:
1854:
1850:
1846:
1838:
1830:
1826:
1822:
1818:
1814:
1810:
1807:(5): 387–97.
1806:
1802:
1795:
1787:
1783:
1779:
1775:
1771:
1767:
1760:
1752:
1748:
1744:
1740:
1737:(3): 277–98.
1736:
1732:
1725:
1717:
1713:
1709:
1705:
1701:
1697:
1694:(2): 138–46.
1693:
1689:
1681:
1672:
1664:
1658:
1643:
1637:
1622:
1618:
1612:
1604:
1600:
1594:
1586:
1582:
1578:
1574:
1567:
1559:
1555:
1551:
1547:
1543:
1539:
1535:
1531:
1523:
1515:
1511:
1507:
1503:
1499:
1495:
1491:
1487:
1483:
1479:
1472:
1464:
1462:
1457:
1451:
1443:
1439:
1437:
1432:
1426:
1418:
1414:
1410:
1406:
1402:
1398:
1394:
1390:
1383:
1375:
1371:
1366:
1361:
1357:
1353:
1349:
1347:
1341:
1335:
1320:
1316:
1310:
1306:
1296:
1295:Proteinopathy
1293:
1292:
1286:
1284:
1280:
1276:
1271:
1267:
1263:
1259:
1255:
1251:
1246:
1242:
1238:
1234:
1224:
1220:
1216:
1212:
1210:
1204:
1195:
1193:
1183:
1180:
1174:
1160:
1157:
1153:
1152:
1151:
1150:
1149:
1140:
1135:
1120:
1117:
1114:
1111:
1108:
1107:
1106:
1105:
1104:
1096:
1092:
1088:
1085:
1082:
1078:
1077:prion protein
1073:
1070:
1067:
1056:
1053:
1045:
1035:
1031:
1027:
1021:
1020:
1016:
1011:This section
1009:
1005:
1000:
999:
986:
984:
980:
976:
972:
968:
964:
960:
956:
951:
948:
946:
943:
939:
935:
931:
927:
923:
919:
918:brain surgery
915:
911:
907:
902:
898:
896:
892:
888:
884:
880:
875:
873:
869:
865:
861:
857:
853:
849:
835:
833:
831:
828:
826:
825:
821:
818:
815:
812:
809:
806:
805:
801:
798:
795:
792:
789:
786:
785:
782:
779:
776:
773:
770:
768:
767:
763:
760:
757:
754:
751:
748:
747:
743:
740:
737:
735:
730:
727:
724:
723:
718:
714:
712:
709:
707:
704:
701:
698:
696:
695:
691:
688:
685:
683:
679:
676:
673:
670:
667:
666:
662:
659:
656:
654:
651:
648:
645:
642:
641:
637:
634:
631:
629:
626:
621:
618:
615:
614:
610:
607:
604:
602:
598:
594:
590:
587:
584:
581:
578:
577:
573:
570:
567:
565:
562:
559:
556:
553:
552:
548:
545:
542:
540:
536:
533:
531:
528:
525:
524:
521:
516:
512:
510:
506:
503:
501:Natural host
500:
498:Disease name
497:
494:
493:
487:
479:
476:
475:sterilization
470:
468:
464:
460:
456:
452:
448:
444:
440:
436:
432:
428:
424:
420:
416:
412:
407:
405:
401:
397:
393:
389:
384:
381:
377:
373:
369:
365:
360:
358:
354:
350:
346:
342:
341:
336:
332:
328:
324:
320:
316:
312:
308:
304:
300:
296:
287:
283:
279:
277:
273:
269:
265:
261:
257:
253:
251:
247:
243:
241:
237:
234:
231:
227:
224:
220:
216:
212:
208:
204:
200:
196:
192:
188:
184:
180:
176:
172:
169:
165:
161:
157:
153:
151:
147:
142:
136:
133:
131:
127:
123:
119:
118:H&E stain
115:
111:
107:
102:
98:
93:
90:Prion disease
89:
85:
80:
68:
58:
54:
52:
46:
43:This article
41:
32:
31:
19:
18:Prion disease
3846:
3822:
3811:
3796:
3781:
3762:
3730:Vorarephilia
3724:
3637:The Americas
3340:
3284:
3256:
3244:
3235:
3221:cite journal
3176:
3172:
3165:Manuelidis L
3159:
3148:. Retrieved
3139:(3): 101–4.
3136:
3132:
3122:
3108:cite journal
3075:
3071:
3064:
3050:cite journal
3009:
3005:
2998:
2984:cite journal
2951:
2947:
2941:
2908:
2904:
2898:
2858:(6680): 11.
2855:
2851:
2841:
2806:
2802:
2792:
2777:
2766:. Retrieved
2757:(3): 101–4.
2754:
2750:
2740:
2729:. Retrieved
2719:
2708:. Retrieved
2704:the original
2694:
2667:
2663:
2650:
2623:
2619:
2606:
2595:. Retrieved
2591:
2582:
2571:. Retrieved
2567:
2558:
2547:. Retrieved
2543:www.anses.fr
2542:
2533:
2522:. Retrieved
2518:
2508:
2497:. Retrieved
2495:. 2019-10-21
2492:
2483:
2472:. Retrieved
2459:
2448:. Retrieved
2441:the original
2436:
2424:
2397:
2393:
2383:
2338:
2334:
2324:
2283:
2279:
2273:
2251:(3): 660–7.
2248:
2244:
2238:
2203:
2199:
2189:
2162:
2158:
2148:
2107:
2103:
2093:
2060:
2056:
2049:
2032:
2028:
2022:
2005:
2001:
1995:
1962:
1958:
1952:
1941:. Retrieved
1937:the original
1927:
1913:{{
1905:{{
1852:
1848:
1837:
1804:
1800:
1794:
1769:
1765:
1759:
1734:
1730:
1724:
1691:
1687:
1680:
1671:
1646:. Retrieved
1636:
1624:. Retrieved
1620:
1611:
1593:
1576:
1572:
1566:
1533:
1529:
1522:
1481:
1477:
1471:
1459:
1450:
1442:the original
1434:
1425:
1392:
1388:
1382:
1355:
1351:
1345:
1334:
1322:. Retrieved
1318:
1309:
1230:
1221:
1217:
1213:
1209:transmission
1205:
1201:
1198:Epidemiology
1189:
1175:
1171:
1146:
1137:
1102:
1093:
1089:
1074:
1063:
1048:
1039:
1024:Please help
1012:
952:
949:
903:
899:
876:
868:histological
852:astrocytosis
845:
682:greater kudu
495:ICTVdb Code
485:
471:
455:conformation
408:
385:
361:
338:
321:, including
302:
298:
294:
293:
240:Risk factors
65:January 2022
62:
48:
44:
3536:Cannibalism
2670:: 198–201.
2626:: 206–207.
2592:www.cdc.gov
2568:www.cdc.gov
1933:"hafci.org"
1395:(1): 1–20.
1346:Spiroplasma
1237:Hippocrates
926:cannibalism
777:vCJD prion
738:Kuru prion
504:Prion name
340:Spiroplasma
159:Usual onset
87:Other names
3824:DiseasesDB
3704:Literature
3687:In fiction
3666:In animals
3357:inherited/
3150:2011-06-20
2768:2011-06-20
2731:2017-11-09
2710:2017-11-09
2597:2017-11-09
2573:2017-11-09
2549:2017-11-09
2524:2024-09-20
2499:2024-07-17
2474:2011-08-19
2450:2011-08-21
2400:: 99–108.
1943:2007-12-02
1772:: 519–50.
1648:2019-03-13
1340:Bastian FO
1301:References
1156:lentivirus
1132:See also:
1042:April 2018
936:people of
879:depression
856:astrocytes
816:FFI prion
796:GSS prion
758:CJD prion
686:EUE prion
657:FSE prion
632:BSE prion
605:CWD prion
568:TME prion
518:Non-human
443:iatrogenic
390:in sheep,
353:microscope
259:Prevention
124:(0.03 mm).
3853:neuro/662
3848:eMedicine
3720:Man-eater
3624:In humans
3423:acquired/
3397:sporadic:
3350:in humans
3289:is being
2890:205000018
2132:1476-4687
2077:0006-291X
1959:Neurology
1915:retracted
1907:retracted
1530:Neurology
1409:1471-8391
1260:in 1921.
1186:Treatment
1168:Diagnosis
1084:mutations
1013:does not
922:autoclave
887:myoclonus
597:mule deer
513:Ruminant
439:aetiology
285:Frequency
276:Prognosis
267:Treatment
130:Specialty
3869:Category
3282:template
3263:and the
3213:17267596
3145:16021982
3100:14230097
2976:23212525
2968:14723996
2763:16021982
2686:15760286
2642:15760286
2416:14522852
2375:17535913
2316:13366031
2308:14593181
2265:12914801
2230:11238871
2181:17923484
2085:12914801
2041:11965173
1979:10563626
1900:39024193
1881:21249178
1829:31976428
1821:11535002
1786:11283320
1716:22600579
1657:cite web
1626:23 April
1585:16823692
1558:25292433
1550:11071481
1417:14522845
1374:17761489
1324:23 April
1289:See also
994:Genetics
963:JC virus
945:ruminant
942:rendered
895:dementia
891:insomnia
601:red deer
423:bacteria
406:prions.
317:of many
150:Symptoms
106:vacuoles
3818:D017096
3673:Poultry
3652:Oceania
3585:Oophagy
3580:Medical
3543:By type
3474:Scrapie
3204:1794316
3181:Bibcode
3092:8598754
3042:4351199
3034:2904126
3014:Bibcode
2913:Bibcode
2905:Science
2882:9590681
2860:Bibcode
2833:9857129
2824:1114482
2366:1887554
2343:Bibcode
2288:Bibcode
2280:Science
2140:8035877
2112:Bibcode
2014:9308333
1987:2915734
1872:3020930
1751:7788281
1708:9266722
1514:4355186
1506:8878476
1486:Bibcode
1254:scrapie
1250:scrapie
1245:scrapie
1227:History
1034:removed
1019:sources
967:CADASIL
864:primate
860:amyloid
848:neurons
530:Scrapie
520:mammals
509:isoform
431:protein
388:scrapie
349:autopsy
319:animals
191:scrapie
122:microns
53:weight.
3678:Spider
3647:Europe
3632:Africa
3614:Sexual
3570:Filial
3301:Curlie
3295:
3286:Curlie
3211:
3201:
3143:
3098:
3090:
3072:Lancet
3040:
3032:
3006:Nature
2974:
2966:
2948:Lancet
2933:142303
2931:
2888:
2880:
2852:Nature
2831:
2821:
2761:
2684:
2640:
2414:
2373:
2363:
2314:
2306:
2263:
2228:
2221:114138
2218:
2179:
2138:
2130:
2104:Nature
2083:
2075:
2039:
2012:
1985:
1977:
1898:
1894:,
1879:
1869:
1827:
1819:
1784:
1749:
1731:Micron
1714:
1706:
1583:
1556:
1548:
1512:
1504:
1478:Nature
1415:
1407:
1372:
983:heroin
975:enzyme
883:ataxia
813:(FFI)
793:(GSS)
755:(CJD)
734:Humans
702:(CSE)
674:(EUE)
649:(FSE)
628:Cattle
585:(CWD)
560:(TME)
459:cattle
451:allele
449:prion
447:mutant
374:, and
345:cortex
335:prions
329:, and
327:cattle
323:humans
307:prions
262:Varies
229:Causes
137:
3829:25165
3595:human
3560:Endo-
3555:Child
3280:‹The
3096:S2CID
3038:S2CID
2972:S2CID
2886:S2CID
2660:(PDF)
2616:(PDF)
2468:(PDF)
2444:(PDF)
2433:(PDF)
2312:S2CID
1983:S2CID
1911:with
1825:S2CID
1712:S2CID
1554:S2CID
1510:S2CID
1283:prion
1266:prion
1081:Point
989:Cause
706:Camel
678:Nyala
622:(BSE)
539:goats
535:Sheep
435:brain
427:prion
419:virus
331:sheep
311:brain
233:Prion
167:Types
51:undue
3813:MeSH
3802:9-CM
3777:8E0Z
3773:8E00
3699:Film
3657:List
3642:Asia
3607:list
3602:Self
3565:Exo-
3442:Kuru
3437:vCJD
3432:iCJD
3404:sCJD
3368:fCJD
3359:PRNP
3339:and
3227:link
3209:PMID
3141:PMID
3114:link
3088:PMID
3056:link
3030:PMID
2990:link
2964:PMID
2929:PMID
2878:PMID
2829:PMID
2759:PMID
2682:PMID
2638:PMID
2412:PMID
2371:PMID
2304:PMID
2261:PMID
2226:PMID
2177:PMID
2136:PMID
2128:ISSN
2081:PMID
2073:ISSN
2037:PMID
2010:PMID
1975:PMID
1896:PMID
1877:PMID
1817:PMID
1782:PMID
1747:PMID
1704:PMID
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