Difference between revisions of "YDL120W"

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|valign="top" nowrap bgcolor="{{SGDblue}}"| '''Systematic name''' || [http://db.yeastgenome.org/cgi-bin/locus.pl?dbid=S000002278 YDL120W]  
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|valign="top" nowrap bgcolor="{{SGDblue}}"| '''Systematic name''' || [http://www.yeastgenome.org/cgi-bin/locus.pl?dbid=S000002278 YDL120W]  
 
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|valign="top" nowrap bgcolor="{{SGDblue}}"| '''Gene name'''        ||''YFH1 ''
 
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'''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<ref name='S000121078'>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 {{SGDpaper|S000121078}} PMID 12732649</ref><ref name='S000117118'>Bulteau AL, et al. (2004) Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science 305(5681):242-5 {{SGDpaper|S000117118}} PMID 15247478</ref><ref name='S000076149'>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 {{SGDpaper|S000076149}} PMID 12947415</ref><ref name='S000075383'>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 {{SGDpaper|S000075383}} PMID 14741370</ref><ref name='S000068798'>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 {{SGDpaper|S000068798}} PMID 12970193</ref><ref name='S000071167'>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 {{SGDpaper|S000071167}} PMID 12221295</ref><ref name='S000057820'>Campuzano V, et al. (1996) Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science 271(5254):1423-7 {{SGDpaper|S000057820}} PMID 8596916</ref><ref name='S000056062'>Rotig A, et al. (1997) Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia. Nat Genet 17(2):215-7 {{SGDpaper|S000056062}} PMID 9326946</ref><ref name='S000050931'>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 {{SGDpaper|S000050931}} PMID 10428860</ref><ref name='S000049863'>Babcock M, et al. (1997) Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin. Science 276(5319):1709-12
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'''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<ref name='S000049863'>Babcock M, et al. (1997) Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin. Science 276(5319):1709-12 {{SGDpaper|S000049863}} PMID 9180083</ref><ref name='S000050931'>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 {{SGDpaper|S000050931}} PMID 10428860</ref><ref name='S000117118'>Bulteau AL, et al. (2004) Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science 305(5681):242-5 {{SGDpaper|S000117118}} PMID 15247478</ref><ref name='S000057820'>Campuzano V, et al. (1996) Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science 271(5254):1423-7 {{SGDpaper|S000057820}} PMID 8596916</ref><ref name='S000071167'>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 {{SGDpaper|S000071167}} PMID 12221295</ref><ref name='S000076149'>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 {{SGDpaper|S000076149}} PMID 12947415</ref><ref name='S000068798'>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 {{SGDpaper|S000068798}} PMID 12970193</ref><ref name='S000121078'>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 {{SGDpaper|S000121078}} PMID 12732649</ref><ref name='S000075383'>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 {{SGDpaper|S000075383}} PMID 14741370</ref><ref name='S000056062'>Rotig A, et al. (1997) Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia. Nat Genet 17(2):215-7
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'''Strain Background''': W303-1B<br>
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'''Strain Background''': W303-1B (''see [[CommunityW303.html|detailed notes]] from Rodney Rothstein and Stephan Bartsch for the W303 strain used in the study'')
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'''Mutation type(s)''': deletion, partial deletion
 
'''Mutation type(s)''': deletion, partial deletion
  

Latest revision as of 07:45, 23 January 2012

Share your knowledge...Edit this entry! <protect>

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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  1. ↑ 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
  2. ↑ 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
  3. ↑ 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
  4. ↑ 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
  5. ↑ 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
  6. ↑ 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
  7. ↑ 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
  8. ↑ 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
  9. ↑ 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
  10. ↑ Rotig A, et al. (1997) Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia. Nat Genet 17(2):215-7 SGD PMID 9326946
  11. ↑ 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
  12. ↑ submitted by Martin Kucej on 2004-03-26

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