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El ensamblaje de gránulos de estrés interrumpe el transporte nucleocitoplasmático
Ke Zhang1, J Gavin Daigle1, Kathleen M Cunningham2
1Department of Neurology, School of Medicine, Johns Hopkins University, Baltimore, MD 21205, USA; Brain Science Institute, School of Medicine, Johns Hopkins University, Baltimore, MD 21205, USA.
Cell
|April 10, 2018
Resumen
El estrés celular interrumpe el transporte nucleocitoplasmático en enfermedades neurodegenerativas como ELA y FTD al formar gránulos de estrés. La inhibición de estos gránulos, por ejemplo, apuntando a Ataxin-2, puede prevenir los defectos de transporte y la neurodegeneración.
Área de la Ciencia:
- La neurociencia
- Biología molecular
- La genética
Sus antecedentes:
- Los defectos de transporte nucleocitoplasmático son clave en la esclerosis lateral amiotrófica (ELA) y la demencia frontotemporal (DFT), a menudo vinculados a las expansiones repetidas de C9ORF72.
- La agregación de proteínas en otras enfermedades neurodegenerativas también sugiere un transporte nucleocitoplasmático interrumpido como un mecanismo patógeno compartido.
Objetivo del estudio:
- Investigar cómo el estrés celular afecta el transporte nucleocitoplasmático en el contexto de la ELA/ETF mediada por C9ORF72.
- Identificar los vínculos moleculares entre los gránulos de estrés y los defectos de transporte nucleocitoplasmático en la neurodegeneración.
Principales métodos:
- Examinó la localización de los factores de transporte nucleocitoplasmático bajo condiciones de estrés celular.
- Investigó el papel de los gránulos de estrés en la interrupción del transporte nucleocitoplasmático.
- Se evaluó el potencial terapéutico de inhibir el ensamblaje de gránulos de estrés (por ejemplo, a través de Ataxina-2 knockdown) en un modelo C9ORF72-ALS/FTD.
Principales resultados:
- El estrés celular hace que los factores críticos de transporte nucleocitoplasmático se acumulen en gránulos de estrés.
- Los gránulos de estrés están directamente implicados en la interrupción del transporte nucleocitoplasmático.
- La inhibición de la formación de gránulos por estrés, específicamente mediante la reducción de los niveles de Ataxina-2, mejora los defectos de transporte nucleocitoplasmático y la neurodegeneración en C9ORF72-ALS/FTD.
Conclusiones:
- Se estableció un vínculo directo entre el ensamblaje de gránulos de estrés y la disfunción de transporte nucleocitoplasmático en la ELA/FTD mediada por C9ORF72.
- Los hallazgos destacan los gránulos de estrés como un objetivo terapéutico crítico para la ELA / FTD y potencialmente otras enfermedades neurodegenerativas que involucran estrés proteico.
- Se ha demostrado que el tratamiento dirigido a Ataxin-2 puede mitigar las características patológicas clave de C9ORF72-ALS/FTD.
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