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TIP Revista Especializada en Ciencias Químico-Biológicas

2021, Número 1

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TIP Rev Esp Cienc Quim Biol 2021; 24 (1)


Viaje al centro de la mitocondria: importación de proteínas, sus alteraciones y enfermedades relacionadas

Avendaño-Monsalve MC, Ponce-Rojas JC, Funes S

Idioma: Español
Referencias bibliográficas: 207
Paginas:
Archivo PDF: 1323.12 Kb.


RESUMEN

Las mitocondrias son organelos fascinantes, no solo porque son el sitio en donde se genera la energía de las células, sino por su relevancia en procesos y patologías de interés médico. La gran mayoría de las proteínas que constituyen el proteoma mitocondrial están codificadas en el núcleo y se traducen por ribosomas citosólicos, por lo que deben ser identificadas correctamente para ser distribuidas e insertadas en cada uno de los subcompartimentos mitocondriales. En este artículo realizamos una descripción de las las maquinarias de importación mitocondrial, además de las diferentes respuestas celulares que contrarrestan las alteraciones en los procesos de transporte de las proteínas o cuando existe una disfunción mitocondrial. Finalmente, mencionamos las enfermedades causadas por mutaciones en los complejos transportadores y de distribución de las proteínas de este organelo, que se han identificado hasta la fecha.


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