Creutzfeldt-Jacob Illness and Other Prion Diseases (Review)

  • José L Colina B Hospital Universitario de Maracaibo, Servicio de Medicina Interna
  • Gabriela Blanchard Hospital Universitario de Maracaibo, Servicio de Pediatría
Keywords: Creutzfeldt-Jakob disease, prions, spongiform encephalopathy

Abstract

Creutzfeldt-Jakob disease (ECJ) is a sub-acute human encephalopathy and progressive associated with a degeneration central nervous system spongiform (SNC). The ECJ belongs to the encephalopathies group transmissible spongiform with an aberrant protein metabolism prions (PrP). Its pathophysiological mechanism exact is still not known clearly but It is thought that this neurodegeneration is related to protein accumulation altered prionics (5, 13, 14, 24, 37). The protein of prions originally identified in rodents infected with Scrapie, it is encoded for a single copy chromosomal gene, this gene is highly conserved and has been identified in more than 13 species of mammals It is usually composed by two exons not translated into 5 separate for a 2 Kb intron. The prevailing uncertainty about PrP and its conformational malleability I dictate the search for linked genes and regulatory elements that could play an active role, but to date it has not been possible to identify any gene related (molecular carbines, etc.), but if some characteristics have been observed not expected within wild genes of PrP.

Published
2003-11-25
How to Cite
1.
Colina B JL, Blanchard G. Creutzfeldt-Jacob Illness and Other Prion Diseases (Review). Kasmera [Internet]. 2003Nov.25 [cited 2024May11];31(2):65-0. Available from: https://www.produccioncientificaluz.org/index.php/kasmera/article/view/4717
Issue
Vol. 31 No. 2 (2003)
Section
Narrative Review

Copyright (c) 2003 José L Colina B, Gabriela Blanchard

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Kasmera journal is registered under a Creative Commons an Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0), available at: https://creativecommons.org/licenses/by-nc-sa/4.0/deed.en; which guarantees the freedom to share-copy and redistribute the material in any medium or format and adapt-remix, transform and build from the material, provided that the name of the authors, the Department of Infectious and Tropical Diseases, Zulia´s University and Kasmera Journal, you must also provide a link to the original document and indicate if changes have been made.

The Department of Infectious and Tropical Diseases, University of Zulia and Kasmera Journal do not retain the rights to published manuscript and the contents are the sole responsibility of the authors, who retain their moral, intellectual, privacy and publicity rights. The guarantee on the intervention of the manuscript (revision, correction of style, translation, layout) and its subsequent dissemination is granted through a license of use and not through a transfer of rights, which represents the Kasmera Journal and Department Infectious Diseases, University of Zulia are exempt from any liability that may arise from ethical misconduct by the authors.

Kasmera is considered a green SHERPA/RoMEO journal, that is, it allows self-archiving of both the pre-print (draft of a manuscript) and the post-print (the corrected and peer-reviewed version) and even the final version (layout as it will be published in the journal) both in personal repositories and in institutional and databases.