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Structure–Function Analysis of Friedreich’s Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe–S Assembly Complex

[Image: see text] Friedreich’s ataxia (FRDA) is a progressive neurodegenerative disease associated with the loss of function of the protein frataxin (FXN) that results from low FXN levels due to a GAA triplet repeat expansion or, occasionally, from missense mutations in the FXN gene. Here biochemica...

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Detalhes bibliográficos
Main Authors: Bridwell-Rabb, Jennifer, Winn, Andrew M., Barondeau, David P.
Formato: Artigo
Idioma:Inglês
Publicado em: 2011
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3340929/
https://ncbi.nlm.nih.gov/pubmed/21776984
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1021/bi200895k
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