The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation

Proceedings of the National Academy of Sciences of the United States of America

Volumn 105, Issue 28, 2008, Pages 9757-9762

  Whitnall, Megan ^a   Rahmanto, Yohan Suryo ^a   Sutak, Robert ^a   Xu, Xiangcong ^a   Becker, Erika M ^a   Mikhael, Marc R ^b   Ponka, Prem ^b   Richardson, Des R ^a  

^a UNIVERSITY OF SYDNEY   (Australia)

^b MCGILL UNIVERSITY   (Canada)

Author keywords

Ferritin; Frataxin; Iron chelators; Mitochondria; Transferrin receptor

Indexed keywords

CD71 ANTIGEN; DEFEROXAMINE; FRATAXIN; PYRIDOXAL ISONICOTINOYLHYDRAZONE; SUCCINATE DEHYDROGENASE; TRANSFERRIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CARDIOMYOPATHY; CONTROLLED STUDY; CYTOSOL; FEMALE; FRIEDREICH ATAXIA; HEART; HEART FUNCTION; HEART MUSCLE; HEART VENTRICLE HYPERTROPHY; IRON CHELATION; IRON DEPLETION; IRON METABOLISM; IRON TRANSPORT; KNOCKOUT MOUSE; MALE; MITOCHONDRION; MOUSE; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN DEPLETION; PROTEIN EXPRESSION;

ANIMALS; BIOLOGICAL TRANSPORT; CARDIOMEGALY; DISEASE MODELS, ANIMAL; FERRITINS; FRIEDREICH ATAXIA; IRON; IRON CHELATING AGENTS; IRON-BINDING PROTEINS; MICE; MICE, KNOCKOUT; MITOCHONDRIA; RECEPTORS, CELL SURFACE;

MUS;

EID: 47749130452     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.0804261105     Document Type: Article

References (33)

1. Campuzano V, et al. (1996) Friedreich's ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science 271:1423-1427.
2. Babcock M, et al. (1997) Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin. Science 276:1709-1712.
3. Rotig A, et al. (1997) Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia. Nat Genet 17:215-217.
4. Wilson RB, Roof DM (1997) Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue. Nat Genet 16:352-357.
5. Puccio H, et al. (2001) Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits. Nat Genet 27:181-186.
6. Richardson DR (2003) Friedreich's ataxia: Iron chelators that target the mitochondrion as a therapeutic strategy? Expert Opin Investig Drugs 12:235-245.
7. Rustin P, et al. (1999) Effect of idebenone on cardiomyopathy in Friedreich's ataxia: A preliminary study. Lancet 354:477-479.
8. Wong A, et al. (1999) The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis. Hum Mol Genet 8:425-430.
9. Richardson DR, Mouralian C, Ponka P, Becker E (2001) Development of potential iron chelators for the treatment of Friedreich's ataxia: Ligands that mobilize mitochondrial iron. Biochim Biophys Acta 1536:133-140.
10. Richardson DR, Ponka P (1998) Pyridoxal isonicotinoyl hydrazone and its analogs: Potential orally effective iron-chelating agents for the treatment of iron overload disease. J Lab Clin Med 131:306-315.
11. Wong CS, Kwok JC, Richardson DR (2004) PCTH: A novel orally active chelator of the aroylhydrazone class that induces iron excretion from mice. Biochim Biophys Acta 1739:70-80.
12. Dunn LL, Suryo-Rahmanto Y, Richardson DR (2007) Iron uptake and metabolism in the new millennium. Trends Cell Biol 17:93-100.
13. Lymboussaki A, et al. (2003) The role of the iron responsive element in the control of ferroportin1/IREG1/MTP1 gene expression. J Hepatol 39:710-715.
14. Levi S, Arosio P (2004) Mitochondrial ferritin. Int J Biochem Cell Biol 36:1887-1889.
15. Meyron-Holtz EG, Ghosh MC, Rouault TA (2004) Mammalian tissue oxygen levels modulate iron-regulatory protein activities in vivo. Science 306:2087-2090.
16. Meyron-Holtz EG, et al. (2004) Genetic ablations of iron regulatory proteins 1 and 2 reveal why iron regulatory protein 2 dominates iron homeostasis. EMBO J 23:386-395.
17. Link G, et al. (2003) Effects of combined chelation treatment with pyridoxal isonicotinoyl hydrazone analogs and deferoxamine in hypertransfused rats and in iron-loaded rat heart cells. Blood 101:4172-4179.
18. Sproule TJ, et al. (2001) Naturally variant autosomal and sex-linked loci determine the severity of iron overload in beta 2-microglobulin-deficient mice. Proc Natl Acad Sci USA 98:5170-5174.
19. Sutak R, et al. (2008) Proteomic analysis of hearts from frataxin knockout mice: Marked rearrangement of energy metabolism, a response to cellular stress and altered expression of proteins involved in cell structure, motility and metabolism. Proteomics 8:1731-1741.
20. Nie G, Sheftel AD, Kim SF, Ponka P (2005) Overexpression of mitochondrial ferritin causes cytosolic iron depletion and changes cellular iron homeostasis. Blood 105:2161-2167.
21. Nie G, Chen G, Sheftel AD, Pantopoulos K, Ponka P (2006) In vivo tumor growth is inhibited by cytosolic iron deprivation caused by the expression of mitochondrial ferritin. Blood 108:2428-2434.
22. Chen OS, Hemenway S, Kaplan J (2002) Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: Evidence that Yfh1p affects Fe-S cluster synthesis. Proc Natl Acad Sci USA 99:12321-12326.
23. Allikmets R, et al. (1999) Mutation of a putative mitochondrial iron transporter gene (ABC7) in X-linked sideroblastic anemia and ataxia (XLSA/A). Hum Mol Genet 8:743-749.
24. Richardson DR, Ponka P, Vyoral D (1996) Distribution of iron in reticulocytes after inhibition of heme synthesis with succinylacetone: Examination of the intermediates involved in iron metabolism. Blood 87:3477-3488.
25. Sheftel AD, Zhang AS, Brown C, Shirihai OS, Ponka P (2007) Direct interorganellar transfer of iron from endosome to mitochondrion. Blood 110:125-132.
26. Greenough M, et al. (2004) Signals regulating trafficking of Menkes (MNK; ATP7A) copper-translocating P-type ATPase in polarized MDCK cells. Am J Physiol Cell Physiol 287:C1463-C1471.
27. Pandolfo M (2003) Friedreich ataxia. Semin Pediatr Neurol 10:163-172.
28. Boddaert N, et al. (2007) Selective iron chelation in Friedreich ataxia: Biologic and clinical implications. Blood 110:401-408.
29. Di Prospero NA, Baker A, Jeffries N, Fischbeck KH (2007) Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: A randomised, placebo-controlled trial. Lancet Neurol 6:878-886.
30. Dunn LL, Sekyere EO, Rahmanto YS, Richardson DR (2006) The function of melanotransferrin: A role in melanoma cell proliferation and tumorigenesis. Carcinogenesis 27:2157-2169.
31. Le NT, Richardson DR (2004) Iron chelators with high antiproliferative activity upregulate the expression of a growth inhibitory and metastasis suppressor gene: A link between iron metabolism and proliferation. Blood 104:2967-2975.
32. Richardson DR, Morgan EH (2004) The transferrin homologue, melanotransferrin, is rapidly catabolised by the liver in the mouse and does not effectively donate iron to the brain. Biochim Biophys Acta 1690:124-133.
33. Babusiak M, Man P, Sutak R, Petrak J, Vyoral D (2005) Identification of heme binding protein complexes in murine erythroleukemic cells: Study by a novel two-dimensional native separation - liquid chromatography and electrophoresis. Proteomics 5:340-350.