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Prions 

Prions

Chapter:
Nomenclature
Author(s):

Harriet S. Meyer

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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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    2. Fauquet CM, Mayo MA, Maniloff J, Desselberger U, Ball LA. Virus Taxonomy: Classification and Nomenclature of Viruses: Eighth Report of the International Committee on Taxonomy of Viruses. San Diego, CA: Elsevier Academic Press; 2005.Find this resource:

      3. Drebot MA, Henchal E, Hjelle B, et al. Improved clarity of meaning from the use of both formal species names and common (vernacular) virus names in virological literature. Arch Virol. 2002;147(12):2465-2471.Find this resource:

      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.

      6. Van Regenmortel MHV, Fauquet CM, Bishop DHL, et al, eds. Virus Taxonomy: Classification and Nomenclature of Viruses: Seventh Report of the International Committee on Taxonomy of Viruses. San Diego, CA: Academic Press; 2000.Find this resource:

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        8. Mayo MA, Fauquet CM, Maniloff J. Taxonomic proposals on the Web: new ICTV consultative procedures. Arch Virol. 2003;148(3):609-611.Find this resource:

        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.Find this resource:

          10. Van Regenmortel MHV, Mahy BWJ. Emerging issues in virus taxonomy. Emerg Infect Dis. 2004;10(1):8-13.Find this resource:

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          13. Van Regenmortel MHV. On the relative merits of italics, Latin and binomial nomenclature in virus taxonomy. Arch Virol. 2000;145(2):433-441.Find this resource:

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          15. Van Regenmortel MHV. How to write the names of virus species. Arch Virol. 1999;144(5):1041-1042.Find this resource:

          16. Calisher CH, Mahy BWJ. Taxonomy: get it right or leave it alone. Am J Trop Med Hyg. 2003;68(5):505-506.Find this resource:

          17. Van Regenmortel MHV. Perspectives on binomial names of virus species. Arch Virol. 2001;146(8):1637-1640.Find this resource:

          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.Find this resource:

            20. Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science. 1982;216(4542):136-144.Find this resource:

            21. Prusiner SB. Prion diseases and the BSE crisis. Science. 1997;278(5336):245-251.Find this resource:

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