| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
J Biol Chem, Vol. 274, Issue 8, 4497-4499, February 19, 1999
From the Department of Pathology, University of Utah,
Salt Lake City, Utah 84112
Mutations in the nuclear gene encoding the
mitochondrial protein frataxin are responsible for the neurological
disorder Friedreich ataxia (FA). Yeast strains with a deletion in the
frataxin homologue YFH1 accumulate excess iron in
mitochondria and demonstrate mitochondrial damage. We show that in the
absence of YFH1, mitochondrial damage is proportional to
the concentration and duration of exposure to extracellular iron,
establishing mitochondrial iron accumulation as causal to mitochondrial
damage. Reintroduction of YFH1 results in the rapid export
of accumulated mitochondrial iron into the cytosol as free, non-heme
bound iron, demonstrating that mitochondrial iron in the yeast FA model
can be made bioavailable. These results demonstrate a mitochondrial
iron cycle in which Yfh1p regulates mitochondrial iron efflux.
This article has been cited by other articles:
|
|
 |
|
  P. R. Anderson, K. Kirby, W. C. Orr, A. J. Hilliker, and J. P. Phillips Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia PNAS, January 15, 2008; 105(2): 611 - 616. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. V. Llorens, J. A. Navarro, M. J. Martinez-Sebastian, M. K. Baylies, S. Schneuwly, J. A. Botella, and M. D. Molto Causative role of oxidative stress in a Drosophila model of Friedreich ataxia FASEB J, February 1, 2007; 21(2): 333 - 344. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. R. Anderson, K. Kirby, A. J. Hilliker, and J. P. Phillips RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila Hum. Mol. Genet., November 15, 2005; 14(22): 3397 - 3405. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Nikali, A. Suomalainen, J. Saharinen, M. Kuokkanen, J. N. Spelbrink, T. Lonnqvist, and L. Peltonen Infantile onset spinocerebellar ataxia is caused by recessive mutations in mitochondrial proteins Twinkle and Twinky Hum. Mol. Genet., October 15, 2005; 14(20): 2981 - 2990. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Campanella, G. Isaya, H. A. O'Neill, P. Santambrogio, A. Cozzi, P. Arosio, and S. Levi The expression of human mitochondrial ferritin rescues respiratory function in frataxin-deficient yeast Hum. Mol. Genet., October 1, 2004; 13(19): 2279 - 2288. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Li and J. Kaplan A Mitochondrial-Vacuolar Signaling Pathway in Yeast That Affects Iron and Copper Metabolism J. Biol. Chem., August 6, 2004; 279(32): 33653 - 33661. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
O. S. Chen, R. J. Crisp, M. Valachovic, M. Bard, D. R. Winge, and J. Kaplan Transcription of the Yeast Iron Regulon Does Not Respond Directly to Iron but Rather to Iron-Sulfur Cluster Biosynthesis J. Biol. Chem., July 9, 2004; 279(28): 29513 - 29518. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. PONKA Hereditary Causes of Disturbed Iron Homeostasis in the Central Nervous System Ann. N.Y. Acad. Sci., March 1, 2004; 1012(1): 267 - 281. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. R. RICHARDSON Novel Chelators for Central Nervous System Disorders That Involve Alterations in the Metabolism of Iron and Other Metal Ions Ann. N.Y. Acad. Sci., March 1, 2004; 1012(1): 326 - 341. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Karthikeyan, J. H. Santos, M. A. Graziewicz, W. C. Copeland, G. Isaya, B. V. Houten, and M. A. Resnick Reduction in frataxin causes progressive accumulation of mitochondrial damage Hum. Mol. Genet., December 15, 2003; 12(24): 3331 - 3342. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. J. Crisp, A. Pollington, C. Galea, S. Jaron, Y. Yamaguchi-Iwai, and J. Kaplan Inhibition of Heme Biosynthesis Prevents Transcription of Iron Uptake Genes in Yeast J. Biol. Chem., November 14, 2003; 278(46): 45499 - 45506. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. D. Alldredge, C. R. Schlieve, N. R. Miller, and L. A. Levin Pathophysiology of the Optic Neuropathy Associated With Friedreich Ataxia Arch Ophthalmol, November 1, 2003; 121(11): 1582 - 1585. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Pastore, G. Tozzi, L. M. Gaeta, E. Bertini, V. Serafini, S. D. Cesare, V. Bonetto, F. Casoni, R. Carrozzo, G. Federici, et al. Actin Glutathionylation Increases in Fibroblasts of Patients with Friedreich's Ataxia: A POTENTIAL ROLE IN THE PATHOGENESIS OF THE DISEASE J. Biol. Chem., October 24, 2003; 278(43): 42588 - 42595. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Luk, M. Carroll, M. Baker, and V. C. Culotta From The Cover: Manganese activation of superoxide dismutase 2 in Saccharomyces cerevisiae requires MTM1, a member of the mitochondrial carrier family PNAS, September 2, 2003; 100(18): 10353 - 10357. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Lesuisse, R. Santos, B. F. Matzanke, S. A. B. Knight, J.-M. Camadro, and A. Dancis Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1) Hum. Mol. Genet., April 15, 2003; 12(8): 879 - 889. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
O. S. Chen, S. Hemenway, and J. Kaplan Genetic analysis of iron citrate toxicity in yeast: Implications for mammalian iron homeostasis PNAS, December 24, 2002; 99(26): 16922 - 16927. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Park, O. Gakh, S. M. Mooney, and G. Isaya The Ferroxidase Activity of Yeast Frataxin J. Biol. Chem., October 4, 2002; 277(41): 38589 - 38595. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Duby, F. Foury, A. Ramazzotti, J. Herrmann, and T. Lutz A non-essential function for yeast frataxin in iron-sulfur cluster assembly Hum. Mol. Genet., October 2, 2002; 11(21): 2635 - 2643. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
O. S. Chen, S. Hemenway, and J. Kaplan Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: Evidence that Yfh1p affects Fe-S cluster synthesis PNAS, September 17, 2002; 99(19): 12321 - 12326. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
U. Muhlenhoff, N. Richhardt, M. Ristow, G. Kispal, and R. Lill The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins Hum. Mol. Genet., August 15, 2002; 11(17): 2025 - 2036. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Foury and T. Roganti Deletion of the Mitochondrial Carrier Genes MRS3 and MRS4 Suppresses Mitochondrial Iron Accumulation in a Yeast Frataxin-deficient Strain J. Biol. Chem., June 28, 2002; 277(27): 24475 - 24483. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Karthikeyan, L. K. Lewis, and M. A. Resnick The mitochondrial protein frataxin prevents nuclear damage Hum. Mol. Genet., May 16, 2002; 11(11): 1351 - 1362. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. M. Becker, J. M. Greer, P. Ponka, and D. R. Richardson Erythroid differentiation and protoporphyrin IX down-regulate frataxin expression in Friend cells: characterization of frataxin expression compared to molecules involved in iron metabolism and hemoglobinization Blood, May 15, 2002; 99(10): 3813 - 3822. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Pilch, E. Jamroz, and E. Marsza Topical Review : Friedreich's Ataxia J Child Neurol, May 1, 2002; 17(5): 315 - 319. [Abstract] [PDF]
|
|
|
|
 |
|
 G Tozzi, M Nuccetelli, M Lo Bello, S Bernardini, L Bellincampi, S Ballerini, L M Gaeta, C Casali, A Pastore, G Federici, et al. Antioxidant enzymes in blood of patients with Friedreich's ataxia Arch. Dis. Child., May 1, 2002; 86(5): 376 - 379. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Cavadini, H. A. O'Neill, O. Benada, and G. Isaya Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia Hum. Mol. Genet., February 1, 2002; 11(3): 217 - 227. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. N. Roy and N. C. Andrews Recent advances in disorders of iron metabolism: mutations, mechanisms and modifiers Hum. Mol. Genet., October 1, 2001; 10(20): 2181 - 2186. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. M. Santos, K. Ohshima, and M. Pandolfo Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm Hum. Mol. Genet., September 1, 2001; 10(18): 1935 - 1944. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. M. Gordon, M. Kogan, S. A.B. Knight, A. Dancis, and D. Pain Distinct roles for two N-terminal cleaved domains in mitochondrial import of the yeast frataxin homolog, Yfh1p Hum. Mol. Genet., February 1, 2001; 10(3): 259 - 269. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. Peyret, M. D. Katinka, S. Duprat, F. Duffieux, V. Barbe, M. Barbazanges, J. Weissenbach, W. Saurin, and C. P. Vivarès Sequence and Analysis of Chromosome I of the Amitochondriate Intracellular Parasite Encephalitozoon cuniculi (Microspora) Genome Res., February 1, 2001; 11(2): 198 - 207. [Abstract] [Full Text]
|
|
|
|
 |
|
 M. A POOK, S. A H AL-MAHDAWI, N. H THOMAS, R. APPLETON, A. NORMAN, R. MOUNTFORD, and S. CHAMBERLAIN Identification of three novel frameshift mutations in patients with Friedreich's ataxia J. Med. Genet., November 1, 2000; 37(11): 38e - 38. [Full Text]
|
|
|
|
|
|
M. Ristow, M. F. Pfister, A. J. Yee, M. Schubert, L. Michael, C.-Y. Zhang, K. Ueki, M. D. Michael II, B. B. Lowell, and C. R. Kahn Frataxin activates mitochondrial energy conversion and oxidative phosphorylation PNAS, October 12, 2000; (2000) 220403797. [Abstract] [Full Text]
|
|
|
|
|
|
P. Cavadini, C. Gellera, P. I. Patel, and G. Isaya Human frataxin maintains mitochondrial iron homeostasis in Saccharomyces cerevisiae Hum. Mol. Genet., October 1, 2000; 9(17): 2523 - 2530. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Voisine, B. Schilke, M. Ohlson, H. Beinert, J. Marszalek, and E. A. Craig Role of the Mitochondrial Hsp70s, Ssc1 and Ssq1, in the Maturation of Yfh1 Mol. Cell. Biol., May 15, 2000; 20(10): 3677 - 3684. [Abstract] [Full Text]
|
|
|
|
|
|
J. M. De Freitas, A. Liba, R. Meneghini, J. S. Valentine, and E. B. Gralla Yeast Lacking Cu-Zn Superoxide Dismutase Show Altered Iron Homeostasis. ROLE OF OXIDATIVE STRESS IN IRON METABOLISM J. Biol. Chem., April 14, 2000; 275(16): 11645 - 11649. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Puccio Recent advances in the molecular pathogenesis of Friedreich ataxia Hum. Mol. Genet., April 1, 2000; 9(6): 887 - 892. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C.-W. Yun, T. Ferea, J. Rashford, O. Ardon, P. O. Brown, D. Botstein, J. Kaplan, and C. C. Philpott Desferrioxamine-mediated Iron Uptake in Saccharomyces cerevisiae. EVIDENCE FOR TWO PATHWAYS OF IRON UPTAKE J. Biol. Chem., March 31, 2000; 275(14): 10709 - 10715. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
O. S. Chen and J. Kaplan CCC1 Suppresses Mitochondrial Damage in the Yeast Model of Friedreich's Ataxia by Limiting Mitochondrial Iron Accumulation J. Biol. Chem., March 10, 2000; 275(11): 7626 - 7632. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. B Delatycki, R. Williamson, and S. M Forrest Friedreich ataxia: an overview J. Med. Genet., January 1, 2000; 37(1): 1 - 8. [Abstract] [Full Text]
|
|
|
|
|
|
J. Li, M. Kogan, S. A. B. Knight, D. Pain, and A. Dancis Yeast Mitochondrial Protein, Nfs1p, Coordinately Regulates Iron-Sulfur Cluster Proteins, Cellular Iron Uptake, and Iron Distribution J. Biol. Chem., November 12, 1999; 274(46): 33025 - 33034. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Pandolfo Molecular Pathogenesis of Friedreich Ataxia Arch Neurol, October 1, 1999; 56(10): 1201 - 1208. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Kaplan Friedreich's ataxia is a mitochondrial disorder PNAS, September 28, 1999; 96(20): 10948 - 10949. [Full Text] [PDF]
|
|
|
|
|
|
Z. L. Harris, A. P. Durley, T. K. Man, and J. D. Gitlin Targeted gene disruption reveals an essential role for ceruloplasmin in cellular iron efflux PNAS, September 14, 1999; 96(19): 10812 - 10817. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Schilke, C. Voisine, H. Beinert, and E. Craig Evidence for a conserved system for iron metabolism in the mitochondria of Saccharomyces cerevisiae PNAS, August 31, 1999; 96(18): 10206 - 10211. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. S. Branda, P. Cavadini, J. Adamec, F. Kalousek, F. Taroni, and G. Isaya Yeast and Human Frataxin Are Processed to Mature Form in Two Sequential Steps by the Mitochondrial Processing Peptidase J. Biol. Chem., August 6, 1999; 274(32): 22763 - 22769. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C.-W. Yun, J. S. Tiedeman, R. E. Moore, and C. C. Philpott Siderophore-Iron Uptake in Saccharomyces cerevisiae. IDENTIFICATION OF FERRICHROME AND FUSARININE TRANSPORTERS J. Biol. Chem., May 19, 2000; 275(21): 16354 - 16359. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Srinivasan, A. Liba, J. A. Imlay, J. S. Valentine, and E. B. Gralla Yeast Lacking Superoxide Dismutase(s) Show Elevated Levels of "Free Iron" as Measured by Whole Cell Electron Paramagnetic Resonance J. Biol. Chem., September 15, 2000; 275(38): 29187 - 29192. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Foury and D. Talibi Mitochondrial Control of Iron Homeostasis. A GENOME WIDE ANALYSIS OF GENE EXPRESSION IN A YEAST FRATAXIN-DEFICIENT STRAIN J. Biol. Chem., March 9, 2001; 276(11): 7762 - 7768. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Li, S. Saxena, D. Pain, and A. Dancis Adrenodoxin Reductase Homolog (Arh1p) of Yeast Mitochondria Required for Iron Homeostasis J. Biol. Chem., January 5, 2001; 276(2): 1503 - 1509. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Li and J. Kaplan The Yeast Gene MSC2, a Member of the Cation Diffusion Facilitator Family, Affects the Cellular Distribution of Zinc J. Biol. Chem., February 9, 2001; 276(7): 5036 - 5043. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Li, O. S. Chen, D. M. Ward, and J. Kaplan CCC1 Is a Transporter That Mediates Vacuolar Iron Storage in Yeast J. Biol. Chem., July 27, 2001; 276(31): 29515 - 29519. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Ristow, M. F. Pfister, A. J. Yee, M. Schubert, L. Michael, C.-Y. Zhang, K. Ueki, M. D. Michael II, B. B. Lowell, and C. R. Kahn Frataxin activates mitochondrial energy conversion and oxidative phosphorylation PNAS, October 24, 2000; 97(22): 12239 - 12243. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. T. Rodriguez-Manzaneque, J. Tamarit, G. Belli, J. Ros, and E. Herrero Grx5 Is a Mitochondrial Glutaredoxin Required for the Activity of Iron/Sulfur Enzymes Mol. Biol. Cell, April 1, 2002; 13(4): 1109 - 1121. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| All ASBMB Journals | Molecular and Cellular Proteomics |
| Journal of Lipid Research | ASBMB Today |
