Normal and Friedreich Ataxia Cells Express Different Isoforms of Frataxin with Complementary Roles in Iron-Sulfur Cluster Assembly

Friedreich ataxia (FRDA) is an autosomal recessive degenerative disease caused by insufficient expression of frataxin (FXN), a mitochondrial iron-binding protein required for Fe-S cluster assembly. The development of treatments to increase FXN levels in FRDA requires elucidation of the steps involve...

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Detalhes bibliográficos
Main Authors: Gakh, Oleksandr, Bedekovics, Tibor, Duncan, Samantha F., Smith, Douglas Y., Berkholz, Donald S., Isaya, Grazia
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2010
Assuntos:
Molecular Bases of Disease
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2992281/
https://ncbi.nlm.nih.gov/pubmed/20889968
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.145144
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