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2008, Number 4

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Enf Infec Microbiol 2008; 28 (4)

Transmissible spongiform encephalopathies and prions

Catalán NJC

Language: Spanish
References: 16
Page: 145-151
PDF size: 117.22 Kb.


ABSTRACT

Transmissible spongiform encephalopathies (TSE) are a group of lethal neurodegenerative diseases in humans and animals, related to a new infectious agent: the prión. These diseases are the result of a conformational change in the PrP protein into a proteases-resistant protein (PrPSc). In humans, these diseases can be divided into three groups: infectious TSE, sporadic TSE and familial or genetic TSE. In 1996, authorities in the United Kingdom discovered the variant of Creutzfeldt-Jakob disease, the human form of the bovine spongiform encephalopathy which can be acquired through ingestion of contaminated meat. Nowadays, there is not an effective drug to stop or delay the damages produced by prións, and this represents a big challenge for the scientific community.


REFERENCES

  1. Chesebro B. “Introduction to the transmissible spongiform encephalopathies or prion diseases”. British Medical Bulletin 2003; 66:1-20.

  2. Rey-Gayo A, Calbo-Torrecilla F. “Estructura de proteínas: plegamiento y priones”. Enferm Infecc Microbiol Clín 2002; 20(4):161-167.

  3. Wadsworth J, Hill A, Beck J, Collinge J. “Molecular and clinical classification of human prion disease”. British Medical Bulletin 2003; 66:241-254.

  4. Sakaguchi S. “Molecular biology of prion protein and its first homologous protein”. The Journal of Medical Investigation 2007; 54:211-223.

  5. Riesner D. “Biochemistry and structure of PrPc and PrPSc”. British Medical Bulletin 2003; 66:21-33.

  6. Lasmézas CI. “Putative functions of PrPc”. British Medical Bulletin 2003; 66:61-70.

  7. Harris DA. “Trafficking, turnover and membrane topology of PrP”. British Medical Bulletin 2003; 66:71-85.

  8. Caughey B. “Prion protein conversions: insight into mechanisms, TSE transmission barriers and strains”. British Medical Bulletin 2003; 66:109-120.

  9. Smith PG, Bradley R. “Bovine spongiform encephalopathy (BSE) and its epidemiology”. British Medical Bulletin 2003; 66:185-198.

  10. Prusiner S. “Prions”. Proc Natl Acad Sci 1998; 95:13363-13383.

  11. Pidone CL. “La teoría del prión”. Analecta Veterinaria 2005; 25(2): 62-72.

  12. Taylor David. “Preventing accidental transmision of human transmisible spongiform encephalopathies”. British Medical Bulletin 2003; 66:293-303.

  13. Dormont D. “Approaches to prophylaxis and therapy”. British Medical Bulletin 2003; 66:281-292.

  14. Kuwata K, Nishida N, Matsumoto T, Kamatari YO, Hosokawa-Muto J, Kodama K et al. “Hot spots in prion protein for pathogenic conversion”. PNAS 2007; 104(29): 11921-11926.

  15. Liebman S. “Progress toward an ultimate proof of the prion hypothesis”. PNAS 2002;99(14): 9098-9100.

  16. CDC Health Information for International Travel 2005-2006. Epidemiology of vCJD and BSE, disponible en www.cdc.gov/travel/yellowBookCh4-VariantPrions.aspx




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