The structure and function of frataxin.
Crit Rev Biochem Mol Biol
; 41(5): 269-91, 2006.
Article
em En
| MEDLINE
| ID: mdl-16911956
ABSTRACT
Frataxin, a highly conserved protein found in prokaryotes and eukaryotes, is required for efficient regulation of cellular iron homeostasis. Humans with a frataxin deficiency have the cardio- and neurodegenerative disorder Friedreich's ataxia, commonly resulting from a GAA trinucleotide repeat expansion in the frataxin gene. While frataxin's specific function remains a point of controversy, the general consensus is that the protein assists in controlling cellular iron homeostasis by directly binding iron. This review focuses on the structural and biochemical aspects of iron binding by the frataxin orthologs and outlines molecular attributes that may help explain the protein's role in different cellular pathways.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Ataxia de Friedreich
/
Modelos Moleculares
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Proteínas de Ligação ao Ferro
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Ferro
Limite:
Animals
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Humans
Idioma:
En
Revista:
Crit Rev Biochem Mol Biol
Ano de publicação:
2006
Tipo de documento:
Article