YDL120W
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| Systematic name | YDL120W |
| Gene name | YFH1 |
| Aliases | |
| Feature type | ORF, Verified |
| Coordinates | Chr IV:245923..246447 |
| Primary SGDID | S000002278 |
Description of YDL120W: 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[1][2][3][4][5][6][7][8][9][10]
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Community Commentary
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Interactions
Genetic
YDL120W is suppressed by RAS2
Strain Background: W303-1B (see detailed notes from Rodney Rothstein and Stephan Bartsch for the W303 strain used in the study)
Mutation type(s): deletion, partial deletion
A mutation in RAS2 supressed a growth defect of deltaYFH1 strain on toxic levels of iron in growth media. [11] [12]
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References
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- ↑ Babcock M, et al. (1997) Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin. Science 276(5319):1709-12 SGD PMID 9180083
- ↑ Branda SS, et al. (1999) Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase. J Biol Chem 274(32):22763-9 SGD PMID 10428860
- ↑ Bulteau AL, et al. (2004) Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science 305(5681):242-5 SGD PMID 15247478
- ↑ Campuzano V, et al. (1996) Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science 271(5254):1423-7 SGD PMID 8596916
- ↑ Chen OS, et al. (2002) Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: evidence that Yfh1p affects Fe-S cluster synthesis. Proc Natl Acad Sci U S A 99(19):12321-6 SGD PMID 12221295
- ↑ Gerber J, et al. (2003) An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1. EMBO Rep 4(9):906-11 SGD PMID 12947415
- ↑ Muhlenhoff U, et al. (2003) Components involved in assembly and dislocation of iron-sulfur clusters on the scaffold protein Isu1p. EMBO J 22(18):4815-25 SGD PMID 12970193
- ↑ Park S, et al. (2003) Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation. J Biol Chem 278(33):31340-51 SGD PMID 12732649
- ↑ Ramazzotti A, et al. (2004) Mitochondrial functional interactions between frataxin and Isu1p, the iron-sulfur cluster scaffold protein, in Saccharomyces cerevisiae. FEBS Lett 557(1-3):215-20 SGD PMID 14741370
- ↑ Rotig A, et al. (1997) Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia. Nat Genet 17(2):215-7 SGD PMID 9326946
- ↑ Kucej M and Foury F (2003) Iron toxicity protection by truncated Ras2 GTPase in yeast strain lacking frataxin. Biochem Biophys Res Commun 310(3):986-91 SGD PMID 14550302
- ↑ submitted by Martin Kucej on 2004-03-26
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