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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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Detalhes bibliográficos
Publicado no:Sci Rep
Main Authors: Nabhan, Joseph F., Gooch, Renea L., Piatnitski Chekler, Eugene L., Pierce, Betsy, Bulawa, Christine E.
Formato: Artigo
Idioma:Inglês
Publicado em: Nature Publishing Group 2015
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4680912/
https://ncbi.nlm.nih.gov/pubmed/26671574
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep18251
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