Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob

The infectious protein or prion (PrPSC) is a transmissible and replicable polypeptide, which arises from an abnormal folding of the PrP protein, by unknown mechanisms and without changes in the primary sequence of its amino acids. Its new spatial disposition arises from the substitution of its alpha...

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Autor principal:	Cartier-Rovirosa,Luis
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2019
Materias:	Creutzfeldt-Jakob Syndrome Prions Prion Diseases
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872019000901176
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spelling	oai:scielo:S0034-988720190009011762020-01-07Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-JakobCartier-Rovirosa,Luis Creutzfeldt-Jakob Syndrome Prions Prion Diseases The infectious protein or prion (PrPSC) is a transmissible and replicable polypeptide, which arises from an abnormal folding of the PrP protein, by unknown mechanisms and without changes in the primary sequence of its amino acids. Its new spatial disposition arises from the substitution of its alpha helices by beta bands, which increase its structural stability, avoiding its complete proteolysis, resulting in a residual accumulation of prions. These prions induce the misfolding of normal PrP protein, generating their exponential increase, leading to a disturbance of neuronal homeostasis which results in the development of the fatal spongiform encephalopathy of the Creutzfeldt-Jakob disease (CJD). This is the most prevalent human prion disease, and 90% of cases are sporadic, suggesting the endogenous genesis of prions. There are different types of prions, identified based on the genetic variance of codon 129 amino acids of the prion protein. Meteonin (M) and Valine (V)), associated with the result of their enzymatic proteolysis, define prions type 1 (21 kDa) and type 2 (19 kDa). The Classical form of CJD produced by MM1 prion occurs in 70% of the cases. The Cerebellar form originated by the VV2 prion occurs in 15% of cases, the form with Kuru plates, associated with the prion MV2 occurs in 5%, and the Vacuolar, related to the MM2 prion occurs in 4%. CJD is always characterized by behavioral, motor, cognitive, and vision alterations and by findings in magnetic resonance imaging, electroencephalogram and cerebrospinal fluid that define each clinical and neuropathological form.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.147 n.9 20192019-09-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872019000901176es10.4067/s0034-98872019000901176
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Creutzfeldt-Jakob Syndrome Prions Prion Diseases
spellingShingle	Creutzfeldt-Jakob Syndrome Prions Prion Diseases Cartier-Rovirosa,Luis Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob
description	The infectious protein or prion (PrPSC) is a transmissible and replicable polypeptide, which arises from an abnormal folding of the PrP protein, by unknown mechanisms and without changes in the primary sequence of its amino acids. Its new spatial disposition arises from the substitution of its alpha helices by beta bands, which increase its structural stability, avoiding its complete proteolysis, resulting in a residual accumulation of prions. These prions induce the misfolding of normal PrP protein, generating their exponential increase, leading to a disturbance of neuronal homeostasis which results in the development of the fatal spongiform encephalopathy of the Creutzfeldt-Jakob disease (CJD). This is the most prevalent human prion disease, and 90% of cases are sporadic, suggesting the endogenous genesis of prions. There are different types of prions, identified based on the genetic variance of codon 129 amino acids of the prion protein. Meteonin (M) and Valine (V)), associated with the result of their enzymatic proteolysis, define prions type 1 (21 kDa) and type 2 (19 kDa). The Classical form of CJD produced by MM1 prion occurs in 70% of the cases. The Cerebellar form originated by the VV2 prion occurs in 15% of cases, the form with Kuru plates, associated with the prion MV2 occurs in 5%, and the Vacuolar, related to the MM2 prion occurs in 4%. CJD is always characterized by behavioral, motor, cognitive, and vision alterations and by findings in magnetic resonance imaging, electroencephalogram and cerebrospinal fluid that define each clinical and neuropathological form.
author	Cartier-Rovirosa,Luis
author_facet	Cartier-Rovirosa,Luis
author_sort	Cartier-Rovirosa,Luis
title	Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob
title_short	Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob
title_full	Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob
title_fullStr	Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob
title_full_unstemmed	Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob
title_sort	enfermedad por priones, encefalopatía espongiforme humana y enfermedad de creutzfeldt-jakob
publisher	Sociedad Médica de Santiago
publishDate	2019
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872019000901176
work_keys_str_mv	AT cartierrovirosaluis enfermedadporprionesencefalopatiaespongiformehumanayenfermedaddecreutzfeldtjakob
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Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob

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