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Conformational stability of human frataxin and effect of Friedreich's ataxia-related mutations on protein folding

The neurodegenerative disorder FRDA (Friedreich's ataxia) results from a deficiency in frataxin, a putative iron chaperone, and is due to the presence of a high number of GAA repeats in the coding regions of both alleles of the frataxin gene, which impair protein expression. However, some FRDA...

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Bibliografiska uppgifter
Huvudupphovsmän: Correia, Ana R., Adinolfi, Salvatore, Pastore, Annalisa, Gomes, Cláudio M.
Materialtyp: Artigo
Språk:Inglês
Publicerad: Portland Press Ltd. 2006
Ämnen:
Länkar:https://ncbi.nlm.nih.gov/pmc/articles/PMC1559467/
https://ncbi.nlm.nih.gov/pubmed/16787388
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1042/BJ20060345
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