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NameFrataxin homolog, mitochondrial
  • Frataxin homolog intermediate form
Gene NameYFH1
Enzyme Class
Biological Properties
General FunctionInvolved in ferroxidase activity
Specific FunctionPromotes the biosynthesis of heme as well as the assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. Plays a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+). Can store large amounts of the metal in the form of a ferrihydrite mineral by oligomerization. May be involved in regulation of the mitochondrial electron transport chain
Cellular LocationMitochondrion matrix
SMPDB PathwaysNot Available
KEGG Pathways
Porphyrin and chlorophyll metabolismec00860 Map00860
SMPDB ReactionsNot Available
KEGG ReactionsNot Available
GO ClassificationNot Available
Gene Properties
Chromosome Locationchromosome 4
Protein Properties
Pfam Domain Function
Protein Residues174
Protein Molecular Weight19490.0
Protein Theoretical pI5.63
Signalling Regions
  • None
Transmembrane Regions
  • None
External Links
Saccharomyces Genome Database YFH1
Uniprot IDQ07540
Uniprot NameFRDA_YEAST
GenBank Gene IDAY558160
Genebank Protein ID45270210
General Reference
  • Jacq, C., Alt-Morbe, J., Andre, B., Arnold, W., Bahr, A., Ballesta, J. P., Bargues, M., Baron, L., Becker, A., Biteau, N., Blocker, H., Blugeon, C., Boskovic, J., Brandt, P., Bruckner, M., Buitrago, M. J., Coster, F., Delaveau, T., del Rey, F., Dujon, B., Eide, L. G., Garcia-Cantalejo, J. M., Goffeau, A., Gomez-Peris, A., Zaccaria, P., et, a. l. .. (1997). "The nucleotide sequence of Saccharomyces cerevisiae chromosome IV." Nature 387:75-78.9169867
  • Hu, Y., Rolfs, A., Bhullar, B., Murthy, T. V., Zhu, C., Berger, M. F., Camargo, A. A., Kelley, F., McCarron, S., Jepson, D., Richardson, A., Raphael, J., Moreira, D., Taycher, E., Zuo, D., Mohr, S., Kane, M. F., Williamson, J., Simpson, A., Bulyk, M. L., Harlow, E., Marsischky, G., Kolodner, R. D., LaBaer, J. (2007). "Approaching a complete repository of sequence-verified protein-encoding clones for Saccharomyces cerevisiae." Genome Res 17:536-543.17322287
  • Babcock, M., de Silva, D., Oaks, R., Davis-Kaplan, S., Jiralerspong, S., Montermini, L., Pandolfo, M., Kaplan, J. (1997). "Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin." Science 276:1709-1712.9180083
  • Radisky, D. C., Babcock, M. C., Kaplan, J. (1999). "The yeast frataxin homologue mediates mitochondrial iron efflux. Evidence for a mitochondrial iron cycle." J Biol Chem 274:4497-4499.9988680
  • Gordon, D. M., Shi, Q., Dancis, A., Pain, D. (1999). "Maturation of frataxin within mammalian and yeast mitochondria: one-step processing by matrix processing peptidase." Hum Mol Genet 8:2255-2262.10545606
  • Branda, S. S., Cavadini, P., Adamec, J., Kalousek, F., Taroni, F., Isaya, G. (1999). "Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase." J Biol Chem 274:22763-22769.10428860
  • Garland, S. A., Hoff, K., Vickery, L. E., Culotta, V. C. (1999). "Saccharomyces cerevisiae ISU1 and ISU2: members of a well-conserved gene family for iron-sulfur cluster assembly." J Mol Biol 294:897-907.10588895
  • Gerber, J., Muhlenhoff, U., Lill, R. (2003). "An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1." EMBO Rep 4:906-911.12947415
  • Ghaemmaghami, S., Huh, W. K., Bower, K., Howson, R. W., Belle, A., Dephoure, N., O'Shea, E. K., Weissman, J. S. (2003). "Global analysis of protein expression in yeast." Nature 425:737-741.14562106
  • Gonzalez-Cabo, P., Vazquez-Manrique, R. P., Garcia-Gimeno, M. A., Sanz, P., Palau, F. (2005). "Frataxin interacts functionally with mitochondrial electron transport chain proteins." Hum Mol Genet 14:2091-2098.15961414
  • Gakh, O., Park, S., Liu, G., Macomber, L., Imlay, J. A., Ferreira, G. C., Isaya, G. (2006). "Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity." Hum Mol Genet 15:467-479.16371422
  • Wang, T., Craig, E. A. (2008). "Binding of yeast frataxin to the scaffold for Fe-S cluster biogenesis, Isu." J Biol Chem 283:12674-12679.18319250
  • Leidgens, S., De Smet, S., Foury, F. (2010). "Frataxin interacts with Isu1 through a conserved tryptophan in its beta-sheet." Hum Mol Genet 19:276-286.19884169
  • Karlberg, T., Schagerlof, U., Gakh, O., Park, S., Ryde, U., Lindahl, M., Leath, K., Garman, E., Isaya, G., Al-Karadaghi, S. (2006). "The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron." Structure 14:1535-1546.17027502
  • Schagerlof, U., Elmlund, H., Gakh, O., Nordlund, G., Hebert, H., Lindahl, M., Isaya, G., Al-Karadaghi, S. (2008). "Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer." Biochemistry 47:4948-4954.18393441