Creutzfeldt-Jacob Illness and Other Prion Diseases (Review)

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...

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Main Authors: José L Colina B, Gabriela Blanchard
Format: Article
Language:English
Published: Universidad del Zulia,Facultad de Medicina,Departamento de Enfermedades Infecciosas y Tropicales 2003-11-01
Series:Kasmera
Subjects:
Online Access:https://produccioncientificaluz.org/index.php/kasmera/article/view/4717
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author José L Colina B
Gabriela Blanchard
author_facet José L Colina B
Gabriela Blanchard
author_sort José L Colina B
collection DOAJ
description 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.
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spelling doaj.art-2ed4015da7054b54b8c1777342fa56af2023-09-02T07:06:34ZengUniversidad del Zulia,Facultad de Medicina,Departamento de Enfermedades Infecciosas y TropicalesKasmera0075-52222477-96282003-11-0131265704716Creutzfeldt-Jacob Illness and Other Prion Diseases (Review)José L Colina B0Gabriela Blanchard1Hospital Universitario de Maracaibo, Servicio de Medicina InternaHospital Universitario de Maracaibo, Servicio de PediatríaCreutzfeldt-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.https://produccioncientificaluz.org/index.php/kasmera/article/view/4717Enfermedad de Creutzfeldt-Jakob, priones, encefalopatía espongiforme
spellingShingle José L Colina B
Gabriela Blanchard
Creutzfeldt-Jacob Illness and Other Prion Diseases (Review)
Kasmera
Enfermedad de Creutzfeldt-Jakob, priones, encefalopatía espongiforme
title Creutzfeldt-Jacob Illness and Other Prion Diseases (Review)
title_full Creutzfeldt-Jacob Illness and Other Prion Diseases (Review)
title_fullStr Creutzfeldt-Jacob Illness and Other Prion Diseases (Review)
title_full_unstemmed Creutzfeldt-Jacob Illness and Other Prion Diseases (Review)
title_short Creutzfeldt-Jacob Illness and Other Prion Diseases (Review)
title_sort creutzfeldt jacob illness and other prion diseases review
topic Enfermedad de Creutzfeldt-Jakob, priones, encefalopatía espongiforme
url https://produccioncientificaluz.org/index.php/kasmera/article/view/4717
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