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ISSN 0016-3813 (Impreso)

2006, Número 5

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Gac Med Mex 2006; 142 (5)


Fisiopatología de las enfermedades por priones

Mandujano A, Montes S, Guzmán A, Espinosa B, Rembao D, Martínez-Cairo S, Zenteno E, Guevara J

Idioma: Español
Referencias bibliográficas: 60
Paginas: 399-406
Archivo PDF: 188.72 Kb.


RESUMEN

Las enfermedades por priones, son trastornos neurodegenerativos progresivos rápidos e invariablemente fatales que afectan tanto a seres humanos como a animales. Tienen formas de presentación esporádica, genética e infecciosa. Los priones son proteínas celulares. No contienen ácidos nucleicos y no son virus o microorganismos. En todos los casos, provocan muerte neuronal, espongiosis común del cerebro, que caracteriza a estas enfermedades, así como agregación de la proteína amiloide prión en forma de placa. La teoría más importante hasta el momento, es la que trata de explicar el cambio de conformación de la proteína prión para producir copias de sí misma y para su agregación y la muerte de las neuronas. Sin embargo, nuevas formas de explicación toman auge actualmente. Una de las más importantes se basa en entender el contenido y cambio de la glicosilación de la proteína prión patológica. Esto permite explicar algunas de sus interacciones, para entender el cambio de conformación y las propiedades físico-químicas de la proteína. Así como algunas de las primeras funciones biológicas (como transportador de iones Cu++2) descritas para esta molécula. En esta revisión abordamos todos los tópicos importantes acerca de estas patologías por demás fascinantes.


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