Prions
15.14.4 Prions
The following are disease names and abbreviations of spongiform encephalopathies2,19-21:
Disease |
Abbreviation |
|---|---|
bovine spongiform encephalopathy (“mad cow disease”) |
BSE |
Creutzfeldt-Jakob disease |
CJD |
familial CJD |
fCJD |
iatrogenic CJD |
iCJD |
sporadic CJD |
sCJD |
variant CJD (formerly new variant CJD [nvCJD]) |
vCJD |
chronic wasting disease of mule deer and elk |
CWD |
exotic ungulate encephalopathy (nyala, greater kudu, oryx) |
EUE |
fatal familial insomnia |
FFI |
feline spongiform encephalopathy |
FSE |
Gerstmann-Sträussler-Scheinker syndrome |
GSS |
kuru |
|
scrapie |
|
transmissible mink encephalopathy |
TME |
transmissible spongiform encephalopathy |
TSE |
(Do not confuse “kudu” and “kuru.”)
The infectious agents of TSEs are known as TSE agents or prions. The term prion (from “proteinaceous infectious particle”) reflects the agents' proposed association or identity with spongiform encephalopathy–related pathologic proteins. Follow author preference for the terms TSE agent and prion.
Proteins related to spongiform encephalopathies in humans are designated as follows:
PrP |
prion protein |
PrP27-30 |
PrP of 27–30 kD |
PrPC |
cellular PrP |
PrPSc |
scrapie-type PrP |
PrP-res |
protease-resistant PrP |
PrP-sen |
protease-sensitive PrP |
rPrP |
recombinant PrP |
BovPrPSc |
(bovine) |
FePrPSc |
(feline) |
HuPrPCJD |
(human) |
HuPrPSc |
(human) |
MDePrPSc |
(mule deer and elk) |
MkPrPSc |
(mink) |
MoPrP |
(mouse) |
NyaPrPSc |
(nyala and greater kudu) |
OvPrPSc |
(ovine [scrapie]) |
Tg(HuPrP) |
(transgenic) |
Tg(MoPrP-P101L) |
The last term refers to a transgenic mouse line with a proline to leucine mutation at residue 101 (see also 15.6.1, Nucleic Acids and Amino Acids).
For prion-related genes, see 15.6.2, Human Gene Nomenclature.
References
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4. Donald Danforth Plant Science Center. ICTVNet. http://www.danforthcenter.org/iltab/ictvnet/asp/_MainPage.asp. Accessed April 21, 2006.
5. Büchen-Osmond C. ICTVdb: The Universal Virus Database of the International Committee on Taxonomy of Viruses. http://phene.cpmc.columbia.edu and http://www.ncbi.nlm.nih.gov/ICTVdb/. Updated February 18, 2005. Accessed April 21, 2006.
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9. Van Regenmortel MHV. Virus nomenclature. In: Maisonneuve H, Enckell PH, Polderman AKS, Thapa R, Vekony M, eds. Science Editors’ Handbook. West Clandon, England: European Association of Science Editors; 2003;§3-4.2:1-4.
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11. Van Regenmortel MHV. Viruses are real, virus species are man-made, taxonomic constructions. Arch Virol. 2003;148(12):2481-2488.
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15. Van Regenmortel MHV. How to write the names of virus species. Arch Virol. 1999;144(5):1041-1042.
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17. Van Regenmortel MHV. Perspectives on binomial names of virus species. Arch Virol. 2001;146(8):1637-1640.
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18. Brunt A, Crabtree K, Dallwitz M, Gibbs A, Watson L, Zurcher E, eds. Plant Viruses Online: Descriptions and Lists From the VIDE Database. http://image.fs.uidaho.edu/vide/refs.htm#names. Accessed December 4, 2006.
19. Asher DM. Transmissible spongiform encephalopathies. In: Murray PR, Baron EJ, Jorgensen JH, Pfaller MA, Yolken RH, eds. Manual of Clinical Microbiology. 8th ed. Washington, DC: ASM Press; 2003:1592-1604.
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20. Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science. 1982;216(4542):136-144.
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21. Prusiner SB. Prion diseases and the BSE crisis. Science. 1997;278(5336):245-251.
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