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Papel de las proteínas de importación nuclear en el mantenimiento de la proteostasis y la patogénesis de enfermedades

Xiuling Zhao¹, Liping Pu², Xuhui Zeng¹

¹Institute of Reproductive Medicine, Medical School, Nantong University, Nantong, Jiangsu 226019, China.

Biochemical pharmacology

|February 22, 2026

Resumen

Los receptores de importación nuclear (NIR) son cruciales para la función celular y la regulación de las proteínas. Los NIR disfuncionales contribuyen a enfermedades como la ELA y el cáncer, pero dirigirse a ellos ofrece nuevas vías terapéuticas.

Área de la Ciencia:

Biología Molecular Biología Molecular
Biología celular Biología celular.
La bioquímica es la bioquímica.

Sus antecedentes:

Los receptores de importación nuclear (NIR), especialmente la importina α/β, regulan el transporte nucleocitoplasmático.
Reconocen las señales de localización nuclear (NLS) para controlar la importación de proteínas en el núcleo.

Objetivo del estudio:

Revisar la base estructural del reconocimiento de NLS por parte de los NIR.
Explorar las funciones de los NIR más allá del transporte, incluida la vigilancia molecular y la regulación co-traducional.
Para discutir el vínculo entre la disfunción de la NIR y las enfermedades humanas.

Principales métodos:

Revisión de la literatura y síntesis de la investigación existente sobre las NIR.
Análisis de datos estructurales y mecanicistas relacionados con el reconocimiento de NLS.
Examen de las consecuencias patológicas y estrategias terapéuticas dirigidas a los NIR.

Principales resultados:

Los NIR reconocen los NLS para importar proteínas esenciales como los factores de transcripción y los factores de reparación del ADN.
Los NIR actúan como acompañantes moleculares, impidiendo la separación de fases aberrante y ayudando al plegamiento de las proteínas.
La disfunción en los NIR está implicada en enfermedades neurodegenerativas (ELA, FTD), cáncer e infecciones virales.

Conclusiones:

Las NIR son vitales para la proteostasis, con funciones que se extienden a la vigilancia molecular y la regulación co-traducional.
El deterioro de la importación nuclear es un factor clave en diversas enfermedades humanas.
La orientación de los NIRs presenta estrategias terapéuticas prometedoras para restaurar la proteostasis y tratar enfermedades, a pesar de los desafíos en cuanto a especificidad y efectos fuera del objetivo.

Palabras clave:

Importado en α/β.El NLS es una NLS.La importación de energía nuclear.La patogénesis y la patogénesis.La proteostasis es la proteostasis.

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