Perturbation of cellular proteostasis networks identifies pathways that modulate precursor and intermediate but not mature levels of frataxin

Friedreich’s Ataxia is a genetic disease caused by expansion of an intronic trinucleotide repeat in the frataxin (FXN) gene yielding diminished FXN expression and consequently disease. Since increasing FXN protein levels is desirable to ameliorate pathology, we explored the role of major cellular pr...

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Detalles Bibliográficos
Autores principales: Nabhan, Joseph F., Gooch, Renea L., Piatnitski Chekler, Eugene L., Pierce, Betsy, Bulawa, Christine E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2015
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4680912/
https://www.ncbi.nlm.nih.gov/pubmed/26671574
http://dx.doi.org/10.1038/srep18251

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4680912/
https://www.ncbi.nlm.nih.gov/pubmed/26671574
http://dx.doi.org/10.1038/srep18251

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