The structure and function of frataxin.

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Bencze KZ, Kondapalli KC, Cook JD, McMahon S, Millan-Pacheco C, Pastor N, Stemmler TL

The structure and function of frataxin.

Crit Rev Biochem Mol Biol. 2006 Sep-Oct;41(5):269-91.

PubMed ID
16911956 [ View in PubMed
]
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.

DrugBank Data that Cites this Article

Drug Targets
DrugTargetKindOrganismPharmacological ActionActions
Ferrous ascorbateFrataxin, mitochondrialProteinHumans
Unknown
Not AvailableDetails
Ferrous fumarateFrataxin, mitochondrialProteinHumans
Unknown
Not AvailableDetails
Ferrous gluconateFrataxin, mitochondrialProteinHumans
Unknown
Not AvailableDetails
Ferrous glycine sulfateFrataxin, mitochondrialProteinHumans
Unknown
Not AvailableDetails
Ferrous succinateFrataxin, mitochondrialProteinHumans
Unknown
Not AvailableDetails
IronFrataxin, mitochondrialProteinHumans
Unknown
Not AvailableDetails