FOG16945
EOG86WWVR

sce:YFH1

Genes: 32

SGD Description
Mitochondrial matrix iron chaperone; oxidizes and stores iron; interacts with Isu1p to promote Fe-S cluster assembly; mutation results in multiple Fe/S-dependent enzyme deficiencies; human frataxin homolog is mutated in Friedrich's ataxia


PomBase Description
frataxin Fxn1


AspGD Description
Ortholog(s) have ferrous iron binding, ferroxidase activity, iron chaperone activity


References

Babcock M, et al. (1997 Jun 13). Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin.

Branda SS, et al. (1999 Aug 6). Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase.

Garland SA, et al. (1999 Dec 10). Saccharomyces cerevisiae ISU1 and ISU2: members of a well-conserved gene family for iron-sulfur cluster assembly.

Radisky DC, et al. (1999 Feb 19). The yeast frataxin homologue mediates mitochondrial iron efflux. Evidence for a mitochondrial iron cycle.

Gordon DM, et al. (1999 Nov). Maturation of frataxin within mammalian and yeast mitochondria: one-step processing by matrix processing peptidase.

Gerber J, et al. (2003 Sep). An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1.

González-Cabo P, et al. (2005 Aug 1). Frataxin interacts functionally with mitochondrial electron transport chain proteins.

Gakh O, et al. (2006 Feb 1). Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity.

Karlberg T, et al. (2006 Oct). The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron.

Schagerlöf U, et al. (2008 Apr 29). Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer.

Wang T, et al. (2008 May 2). Binding of yeast frataxin to the scaffold for Fe-S cluster biogenesis, Isu.

Leidgens S, et al. (2010 Jan 15). Frataxin interacts with Isu1 through a conserved tryptophan in its beta-sheet.

Mitochondrial localization predictions
Predotar	TargetP	MitoProt
Raw data
Phobius transmembrane predictions 0 genes with posterior transmembrane prediction > 50%