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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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Main Authors: | , , , |
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Formato: | Artigo |
Idioma: | Inglês |
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Portland Press Ltd.
2006
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Acceso en liña: | 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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