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Biophysical characterisation of the recombinant human frataxin precursor

Friedreich's ataxia is a disease caused by a decrease in the levels of expression or loss of functionality of the mitochondrial protein frataxin (FXN). The development of an active and stable recombinant variant of FXN is important for protein replacement therapy. Although valuable data about the ma...

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Autores principales: Ignacio Hugo Castro, Alejandro Ferrari, María Georgina Herrera, Martín Ezequiel Noguera, Lorenzo Maso, Monica Benini, Alessandra Rufini, Roberto Testi, Paola Costantini, Javier Santos
Formato: Artigo
Lenguaje:Inglês
Publicado: Wiley 2018-03-01
Colección:FEBS Open Bio
Materias:
aggregation
conformation
Friedreich's ataxia
precursor
stability
unfolding
Acceso en línea:https://doi.org/10.1002/2211-5463.12376
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