関: variant Creutzfeldt-Jakob disease

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/08/20 19:27:43」(JST)

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Variant Creutzfeldt–Jakob disease (vCJD) or new variant Creutzfeldt–Jakob disease (nvCJD) is a transmissible spongiform encephalopathy which was identified in 1996 by the National CJD Surveillance Unit in Edinburgh, Scotland. It is an often fatal type of food poisoning caused by prions. Over 170 cases of vCJD have been recorded in the United Kingdom, and around 30 cases in the rest of the world. The fact that the epidemiology of the disease coincided with an epidemic of bovine spongiform encephalopathy led to the hypothesis that consumption of BSE-infected beef caused the disease.^[1]^[2] It is a different disease from Creutzfeldt–Jakob disease.

Despite the consumption of contaminated beef in the UK being reckoned to be quite high, vCJD has infected a comparatively small cohort of people. One explanation for this can be found in the genetics of patients with the disease. The human PRNP protein which is subverted in prion disease can occur with either methionine or valine at amino acid 129, without any apparent difference in normal function. Of the overall Caucasian population, about 40% have two methionine-containing alleles, 10% have two valine-containing alleles, and the other 50% are heterozygous at this position. Only a single vCJD patient tested was found to be heterozygous; most of those affected had two copies of the methionine-containing form. Additionally, for unknown reasons, those affected are generally under the age of 40. It is not yet known whether those unaffected are actually immune or only have a longer incubation period until symptoms appear.^[3]^[4]

References

^ Ironside, JW (Jul 2010). "Variant Creutzfeldt–Jakob disease.". Haemophilia. 16 Suppl 5: 175–80. PMID 20590878. doi:10.1111/j.1365-2516.2010.02317.x.
^ Ironside, JW (2012). "Variant Creutzfeldt–Jakob disease: an update.". Folia Neuropathologica. 50 (1): 50–6. PMID 22505363.
^ Saba, R; Booth, SA (2013). "The genetics of susceptibility to variant Creutzfeldt–Jakob disease.". Public health genomics. 16 (1-2): 17–24. PMID 23548713. doi:10.1159/000345203.
^ Sikorska, B; Liberski, PP (2012). "Human prion diseases: from Kuru to variant Creutzfeldt–Jakob disease.". Sub-cellular biochemistry. 65: 457–96. PMID 23225013. doi:10.1007/978-94-007-5416-4_17.

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1. 変異型クロイツフェルト・ヤコブ病 variant creutzfeldt jakob disease
2. クロイツフェルト・ヤコブ病 creutzfeldt jakob disease
3. プリオンによる中枢神経系疾患 diseases of the central nervous system caused by prions
4. 供血者スクリーニング：病歴 blood donor screening medical history
5. プリオンの生物学および遺伝学 biology and genetics of prions

English Journal

Infectivity in bone marrow from sporadic CJD patients.

Huor A1, Douet JY1, Lacroux C1, Lugan S1, Tillier C1, Aron N1, Cassard H1, Arnold M2, Torres JM3, Ironside JW4, Andréoletti O1.
The Journal of pathology.J Pathol.2017 Aug 9. doi: 10.1002/path.4954. [Epub ahead of print]
PMID 28791720

Creutzfeldt-Jakob disease lookback study: 21 years of surveillance for transfusion transmission risk.

Crowder LA1, Schonberger LB2, Dodd RY3, Steele WR1.
Transfusion.Transfusion.2017 Aug;57(8):1875-1878. doi: 10.1111/trf.14145. Epub 2017 Apr 25.
PMID 28444687

Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients.

Douet JY, Lacroux C, Aron N, Head MW, Lugan S, Tillier C, Huor A, Cassard H, Arnold M, Beringue V, Ironside JW, Andréoletti O.
Emerging infectious diseases.Emerg Infect Dis.2017 Jun;23(6):946-956. doi: 10.3201/eid2306.161734.
In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study, we used in vitro amplification of prions by protein misfol
PMID 28518033