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Las encefalopatías espongiformes transmisibles
Steven J Collins1, Victoria A Lawson, Colin L Masters
1Australian National Creutzfeldt-Jakob Disease Registry, Melbourne, Australia. stevenjc@unimelb.edu.au
Lancet (London, England)
|January 16, 2004
Resumen
Las encefalopatías espongiformes transmisibles (ETS), o enfermedades priónicas, comparten causas con el Alzheimer y el Parkinson, pero son distintas debido a la transmisibilidad de las proteínas. La vigilancia contra los reservorios potenciales de infectividad es crucial para prevenir futuras epidemias.
Área de la Ciencia:
- La neurociencia es la neurociencia.
- Patología Patología Patología.
- Enfermedades infecciosas Enfermedades infecciosas.
Sus antecedentes:
- Las encefalopatías espongiformes transmisibles (ETS), también conocidas como enfermedades priónicas, son trastornos neurodegenerativos.
- Comparten un mecanismo patógeno común con las enfermedades de Alzheimer y Parkinson: la ganancia tóxica de la función de las proteínas aberrantes.
- Las EET son únicas debido a la transmisibilidad de la estructura proteica anormal.
Objetivo del estudio:
- Clasificar las EET nosológicamente con otros trastornos neurodegenerativos.
- Explorar los mecanismos patógenos y la transmisibilidad de las enfermedades priónicas.
- Reevaluar las barreras de las especies y el control de las infecciones a la luz de nuevas evidencias.
Principales métodos:
- Análisis comparativo de los mecanismos de las enfermedades neurodegenerativas.
- Revisión de los datos epidemiológicos sobre los brotes de EET (por ejemplo, encefalopatía espongiforme bovina, kuru).
- Evaluación de la evidencia de infecciones crónicas subclínicas en animales.
Principales resultados:
- Las EET se agrupan nosológicamente con la enfermedad de Alzheimer y la enfermedad de Parkinson, que difieren principalmente en la transmisibilidad.
- La estructura proteica anormal es clave para la transmisibilidad de las EET.
- Las epidemias surgen de un reciclaje intraespecífico sostenido de la proteína anormal.
- Las infecciones crónicas subclínicas en animales informan la patogénesis y desafían las nociones existentes de barreras entre especies.
Conclusiones:
- Las EET y otras enfermedades neurodegenerativas provienen del mal plegamiento de las proteínas y del deterioro del aclaramiento.
- La transmisibilidad, inherente a la estructura de las proteínas priónicas, distingue a las EET.
- El reciclaje sostenido de proteínas puede conducir a grandes epidemias, lo que requiere una vigilancia continua.
- La vigilancia continua de los reservorios de infectividad es esencial para prevenir las epidemias de EET.
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