Balancing acts: Molecular control of mammalian iron metabolism

Cell

Volumn 117, Issue 3, 2004, Pages 285-297

  Hentze, Matthias W ^a   Muckenthaler, Martina U ^b   Andrews, Nancy C ^c  

^a EUROPEAN MOLECULAR BIOLOGY LABORATORY   (Germany)

^b UNIVERSITY OF HEIDELBERG   (Germany)

^c HOWARD HUGHES MEDICAL INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

DEGENERATIVE DISEASE; GENE CONTROL; HEMATOLOGIC DISEASE; HOMEOSTASIS; IMMUNE SYSTEM; IRON METABOLISM; METABOLIC DISORDER; MOLECULAR DYNAMICS; NONHUMAN; PRIORITY JOURNAL; REVIEW;

MAMMALIA;

EID: 2042546096     PISSN: 00928674     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0092-8674(04)00343-5     Document Type: Review

References (103)

1. Abboud S., Haile D.J. A novel mammalian iron-regulated protein involved in intracellular iron metabolism. J. Biol. Chem. 275:2000;19906-19912
2. Adamec J., Rusnak F., Owen W.G., Naylor S., Benson L.M., Gacy A.M., Isaya G. Iron-dependent self-assembly of recombinant yeast frataxin. implications for Friedreich ataxia Am. J. Hum. Genet. 67:2000;549-562. Published online August 4, 2000.
3. Ahmad K.A., Ahmann J.R., Migas M.C., Waheed A., Britton R.S., Bacon B.R., Sly W.S., Fleming R.E. Decreased liver hepcidin expression in the hfe knockout mouse. Blood Cells Mol. Dis. 29:2002;361-366
4. Allikmets R., Raskind W.H., Hutchinson A., Schueck N.D., Dean M., Koeller D.M. Mutation of a putative mitochondrial iron transporter gene (ABC7) in X-linked sideroblastic anemia and ataxia (XLSA/A). Hum. Mol. Genet. 8:1999;743-749
5. Beard J. Iron deficiency alters brain development and functioning. J. Nutr. 133:2003;1468-1472
6. Bennett M.J., Lebron J.A., Bjorkman P.J. Crystal structure of the hereditary haemochromatosis protein HFE complexed with transferrin receptor. Nature. 403:2000;46-53
7. Bourdon E., Kang D.K., Ghosh M.C., Drake S.K., Wey J., Levine R.L., Rouault T.A. The role of endogenous heme synthesis and degradation domain cysteines in cellular iron-dependent degradation of IRP2. Blood Cells Mol. Dis. 31:2003;247-255
8. Bridle K.R., Frazer D.M., Wilkins S.J., Dixon J.L., Purdie D.M., Crawford D.H., Subramaniam V.N., Powell L.W., Anderson G.J., Ram G.A. Disrupted hepcidin regulation in HFE-associated haemochromatosis and the liver as a regulator of body iron homoeostasis. Lancet. 361:2003;669-673
9. Brittenham G.M. The red cell cycle. Brock J.H., et al. Iron Metabolism in Health and Disease. 1994;31-62 W.B. Saunders Company Ltd, London
10. Brown N.M., Anderson S.A., Steffen D.W., Carpenter T.B., Kennedy M.C., Walden W.E., Eisenstein R.S. Novel role of phosphorylation in Fe-S cluster stability revealed by phosphomimetic mutations at Ser-138 of iron regulatory protein 1. Proc. Natl. Acad. Sci. USA. 95:1998;15235-15240
11. Buys S.S., Martin C.B., Eldridge M., Kushner J.P., Kaplan J. Iron absorption in hypotransferrinemic mice. Blood. 78:1991;3288-3290
12. Camaschella C., Roetto A., Cali A., De Gobbi M., Garozzo G., Carella M., Majorano N., Totaro A., Gasparini P. The gene TFR2 is mutated in a new type of haemochromatosis mapping to 7q22. Nat. Genet. 25:2000;14-15
13. Canonne-Hergaux F., Levy J.E., Fleming M.D., Montross L.K., Andrews N.C., Gros P. Expression of the DMT1 (NRAMP2/DCT1) iron transporter in mice with genetic iron overload disorders. Blood. 97:2001;1138-1140
14. Cazzola M., Skoda R.C. Translational pathophysiology. a novel molecular mechanism of human disease Blood. 95:2000;3280-3288
15. Chen O.S., Hemenway S., Kaplan J. Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export. evidence that Yfh1p affects Fe-S cluster synthesis Proc. Natl. Acad. Sci. USA. 99:2002;12321-12326
16. Cheng Y., Zak O., Aisen P., Harrison S.C., Walz T. Structure of the human transferrin receptor-transferrin complex. Cell. 116:2004;565-576
17. Courselaud B., Pigeon C., Inoue Y., Inoue J., Gonzalez F.J., Leroyer P., Gilot D., Boudjema K., Guguen-Guillouzo C., Brissot P., et al. C/EBPalpha regulates hepatic transcription of hepcidin, an antimicrobial peptide and regulator of iron metabolism. Cross-talk between C/EBP pathway and iron metabolism. J. Biol. Chem. 277:2002;41163-41170
18. Curtis A.R., Fey C., Morris C.M., Bindoff L.A., Ince P.G., Chinnery P.F., Coulthard A., Jackson M.J., Jackson A.P., McHale D.P., et al. Mutation in the gene encoding ferritin light polypeptide causes dominant adult-onset basal ganglia disease. Nat. Genet. 28:2001;350-354
19. De Sousa M., Breedvelt F., Dynesius-Trentham R., Trentham D., Lum J. Iron, iron-binding proteins and immune system cells. Ann. N Y Acad. Sci. 526:1988;310-322
20. Donovan A., Brownlie A., Zhou Y., Shepard J., Pratt S.J., Moynihan J., Paw B.H., Drejer A., Barut B., Zapata A., et al. Positional cloning of zebrafish ferroportin1 identifies a conserved vertebrate iron exporter. Nature. 403:2000;776-781
21. Eisenstein R.S., Ross K.L. Novel roles for iron regulatory proteins in the adaptive response to iron deficiency. J. Nutr. 133:2003;1510-1516
22. Feder J.N., Gnirke A., Thomas W., Tsuchihashi Z., Ruddy D.A., Basava A., Dormishian F., Domingo R. Jr., Ellis M.C., Fullan A., et al. A novel MHC class I-like gene is mutated in patients with hereditary haemochromatosis. Nat. Genet. 13:1996;399-408
23. Ferguson C.J., Wareing M., Ward D.T., Green R., Smith C.P., Riccardi D. Cellular localization of divalent metal transporter DMT-1 in rat kidney. Am. J. Physiol. Renal Physiol. 280:2001;F803-F814
24. Fillebeen C., Chahine D., Caltagirone A., Segal P., Pantopoulos K. A phosphomimetic mutation at Ser-138 renders iron regulatory protein 1 sensitive to iron-dependent degradation. Mol. Cell. Biol. 23:2003;6973-6981
25. Finch C. Regulators of iron balance in humans. Blood. 84:1994;1697-1702
26. Fleming M.D., Trenor C.C. 3rd, Su M.A., Foernzler D., Beier D.R., Dietrich W.F., Andrews N.C. Microcytic anaemia mice have a mutation in Nramp2, a candidate iron transporter gene. Nat. Genet. 16:1997;383-386
27. Fleming M.D., Romano M.A., Su M.A., Garrick L.M., Garrick M.D., Andrews N.C. Nramp2 is mutated in the anemic Belgrade (b) rat. evidence of a role for Nramp2 in endosomal iron transport Proc. Natl. Acad. Sci. USA. 95:1998;1148-1153
28. Gerber J., Lill R. Biogenesis of iron-sulfur proteins in eukaryotes. components, mechanism and pathology Mitochondrion. 2:2002;71-86
29. Gerber J., Muhlenhoff U., Lill R. An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1. EMBO Rep. 4:2003;906-911
30. Gross C.N., Irrinki A., Feder J.N., Enns C.A. Co-trafficking of HFE, a nonclassical major histocompatibility complex class I protein, with the transferrin receptor implies a role in intracellular iron regulation. J. Biol. Chem. 273:1998;22068-22074
31. Gunshin H., Mackenzie B., Berger U.V., Gunshin Y., Romero M.F., Boron W.F., Nussberger S., Gollan J.L., Hediger M.A. Cloning and characterization of a mammalian proton-coupled metal-ion transporter. Nature. 388:1997;482-488
32. Gunshin H., Allerson C.R., Polycarpou-Schwarz M., Rofts A., Rogers J.T., Kishi F., Hentze M.W., Rouault T.A., Andrews N.C., Hediger M.A. Iron-dependent regulation of the divalent metal ion transporter. FEBS Lett. 509:2001;309-316
33. Halliwell B. Role of free radicals in the neurodegenerative diseases. therapeutic implications for antioxidant treatment Drugs Aging. 18:2001;685-716
34. Hanson E.S., Rawlins M.L., Leibold E.A. Oxygen and iron regulation of iron regulatory protein 2. J. Biol. Chem. 278:2003;40337-40342
35. Harris Z.L., Durley A.P., Man T.K., Gitlin J.D. Targeted gene disruption reveals an essential role for ceruloplasmin in cellular iron efflux. Proc. Natl. Acad. Sci. USA. 96:1999;10812-10817
36. Harrison P.M. Ferritin. an iron-storage molecule Semin. Hematol. 14:1977;55-70
37. Hanson E.S., Foot L.M., Leibold E.A. Hypoxia post-translationally activates iron-regulatory protein 2. J. Biol. Chem. 274:1999;5047-5052
38. Hentze M.W., Kühn L.C. Molecular control of vertebrate iron metabolism. mRNA-based regulatory circuits operated by iron, nitric oxide, and oxidative stress Proc. Natl. Acad. Sci. USA. 93:1996;8175-8182
39. Hentze M.W., Caughman S.W., Casey J.L., Koeller D.M., Rouault T.A., Harford J.B., Klausner R.D. A model for the structure and functions of iron-responsive elements. Gene. 72:1988;201-208
40. Hubert N., Hentze M.W. Previously uncharacterized isoforms of divalent metal transporter (DMT)-1. implications for regulation and cellular function Proc. Natl. Acad. Sci. USA. 99:2002;12345-12350
41. Jeong S.Y., David S. Glycosylphosphatidylinositol-anchored ceruloplasmin is required for iron efflux from cells in the central nervous system. J. Biol. Chem. 278:2003;27144-27148
42. Kang D.K., Jeong J., Drake S.K., Wehr N.B., Rouault T.A., Levine R.L. Iron regulatory protein 2 as iron sensor. Iron-dependent oxidative modification of cysteine. J. Biol. Chem. 278:2003;14857-14864
43. Kato J., Fujikawa K., Kanda M., Fukuda N., Sasaki K., Takayama T., Kobune M., Takada K., Takimoto R., Hamada H., et al. A mutation, in the iron-responsive element of H ferritin mRNA, causing autosomal dominant iron overload. Am. J. Hum. Genet. 69:2001;191-197
44. Kawabata H., Yang R., Hirama T., Vuong P.T., Kawano S., Gombart A.F., Koeffler H.P. Molecular cloning of transferrin receptor 2. A new member of the transferrin receptor-like family. J. Biol. Chem. 274:1999;20826-20832
45. Kozyraki R., Fyfe J., Verroust P.J., Jacobsen C., Dautry-Varsat A., Gburek J., Willnow T.E., Christensen E.I., Moestrup S.K. Megalin-dependent cubilin-mediated endocytosis is a major pathway for the apical uptake of transferrin in polarized epithelia. Proc. Natl. Acad. Sci. USA. 98:2001;12491-12496
46. Krause A., Neitz S., Magert H.J., Schulz A., Forssmann W.G., Schulz-Knappe P., Adermann K. LEAP-1, a novel highly disulfide-bonded human peptide, exhibits antimicrobial activity. FEBS Lett. 480:2000;147-150
47. Kristiansen M., Graversen J.H., Jacobsen C., Sonne O., Hoffman H.J., Law S.K., Moestrup S.K. Identification of the haemoglobin scavenger receptor. Nature. 409:2001;198-201
48. Laftah, A.H., Ramesh, B., Simpson, R.J., Solanky, N., Bahram, S., Schumann, K., Debnam, E.S., and Srai, S.K. (2004). Effect of hepcidin on intestinal iron absorption in mice. Blood, published online January 29, 2004. 10.1182/blood-2003-03-0953.
49. LaVaute T., Smith S., Cooperman S., Iwai K., Land W., Meyron-Holtz E., Drake S.K., Miller G., Abu-Asab M., Tsokos M., et al. Targeted deletion of the gene encoding iron regulatory protein-2 causes misregulation of iron metabolism and neurodegenerative disease in mice. Nat. Genet. 27:2001;209-214
50. Lebron J.A., Bennett M.J., Vaughn D.E., Chirino A.J., Snow P.M., Mintier G.A., Feder J.N., Bjorkman P.J. Crystal structure of the hemochromatosis protein HFE and characterization of its interaction with transferrin receptor. Cell. 93:1998;111-123
51. Levi S., Corsi B., Bosisio M., Invernizzi R., Volz A., Sanford D., Arosio P., Drysdale J. A human mitochondrial ferritin encoded by an intronless gene. J. Biol. Chem. 276:2001;24437-24440
52. Levy J.E., Jin O., Fujiwara Y., Kuo F., Andrews N.C. Transferrin receptor is necessary for development of erythrocytes and the nervous system. Nat. Genet. 21:1999;396-399
53. Levy J.E., Montross L.K., Andrews N.C. Genes that modify the hemochromatosis phenotype in mice. J. Clin. Invest. 105:2000;1209-1216
54. Liu X.B., Hill P., Haile D.J. Role of the ferroportin iron-responsive element in iron and nitric oxide dependent gene regulation. Blood Cells Mol. Dis. 29:2002;315-326
55. Ludwiczek S., Aigner E., Theurl I., Weiss G. Cytokine mediated regulation of iron transport in human monocytic cells. Blood. 101:2003;4148-4154
56. Mazumder B., Sampath P., Seshadri V., Maitra R.K., DiCorleto P.E., Fox P.L. Regulated release of L13a from the 60S ribosomal subunit as a mechanism of transcript-specific translational control. Cell. 115:2003;187-198
57. McKie A.T., Marciani P., Rolfs A., Brennan K., Wehr K., Barrow D., Miret S., Bomford A., Peters T.J., Farzaneh F., et al. A novel duodenal iron-regulated transporter, IREG1, implicated in the basolateral transfer of iron to the circulation. Mol. Cell. 5:2000;299-309
58. McKie A.T., Barrow D., Latunde-Dada G.O., Rolfs A., Sager G., Mudaly E., Mudaly M., Richardson C., Barlow D., Bomford A., et al. An iron-regulated ferric reductase associated with the absorption of dietary iron. Science. 291:2001;1755-1759
59. Meyron-Holtz E.G., Vaisman B., Cabantchik Z.I., Fibach E., Rouault T.A., Hershko C., Konijn A.M. Regulation of intracellular iron metabolism in human erythroid precursors by internalized extracellular ferritin. Blood. 94:1999;3205-3211
60. Meyron-Holtz E.G., Ghosh M.C., Iwai K., LaVaute T., Brazzolotto X., Berger U.V., Land W., Ollivierre-Wilson H., Grinberg A., Love P., Rouault T.A. Genetic ablations of iron regulatory proteins 1 and 2 reveal why iron regulatory protein 2 dominates iron homeostasis. EMBO J. 23:2004;386-395. Published online January 15, 2004. doi: 10.1038/sj.emboj.7600041
61. Miyajima H., Kono S., Takahashi Y., Sugimoto M. Increased lipid peroxidation and mitochondrial dysfunction in aceruloplasminemia brains. Blood Cells Mol. Dis. 29:2002;433-438
62. Montosi G., Donovan A., Totaro A., Garuti C., Pignatti E., Cassanelli S., Trenor C.C., Gasparini P., Andrews N.C., Pietrangelo A. Autosomal-dominant hemochromatosis is associated with a mutation in the ferroportin (SLC11A3) gene. J. Clin. Invest. 108:2001;619-623
63. Muckenthaler M., Gray N.K., Hentze M.W. IRP-1 binding to ferritin mRNA prevents the recruitment of the small ribosomal subunit by the cap-binding complex eIF4F. Mol. Cell. 2:1998;383-388
64. Muckenthaler M., Roy C.N., Custodio A.O., Minana B., deGraaf J., Montross L.K., Andrews N.C., Hentze M.W. Regulatory defects in liver and intestine implicate abnormal hepcidin and Cybrd1 expression in mouse hemochromatosis. Nat. Genet. 34:2003;102-107
65. Muhlenhoff U., Gerber J., Richhardt N., Lill R. Components involved in assembly and dislocation of iron-sulfur clusters on the scaffold protein Isu1p. EMBO J. 22:2003;4815-4825
66. Mukhopadhyay C.K., Attieh Z.K., Fox P.L. Role of ceruloplasmin in cellular iron uptake. Science. 279:1998;714-717
67. Mwanjewe J., Grover A.K. Role of transient receptor potential canonical 6 (TRPC6) in non-transferrin-bound iron uptake in neuronal phenotype PC12 cells. Biochem. J. 378:2004;975-982
68. Nemeth E., Valore E.V., Territo M., Schiller G., Lichtenstein A., Ganz T. Hepcidin, a putative mediator of anemia of inflammation, is a type II acute-phase protein. Blood. 101:2003;2461-2463
69. Nemeth, E., Rivera, S., Gabayan, V., Keller, C., Taudorf, S., Pedersen, B.K., and Ganz, T. (2004). Interleukin-6 mediates hypoferremia of inflammation by inducing the synthesis of the iron-regulatory hormone hepcidin. J. Clin. Invest., in press.
70. Nicolas G., Bennoun M., Devaux I., Beaumont C., Grandchamp B., Kahn A., Vaulont S. Lack of hepcidin gene expression and severe tissue iron overload in upstream stimulatory factor 2 (USF2) knockout mice. Proc. Natl. Acad. Sci. USA. 98:2001;8780-8785
71. Nicolas G., Bennoun M., Porteu A., Mativet S., Beaumont C., Grandchamp B., Sirito M., Sawadogo M., Kahn A., Vaulont S. Severe iron deficiency anemia in transgenic mice expressing liver hepcidin. Proc. Natl. Acad. Sci. USA. 99:2002;4596-4601. a
72. Nicolas G., Chauvet C., Viatte L., Danan J.L., Bigard X., Devaux I., Beaumont C., Kahn A., Vaulont S. The gene encoding the iron regulatory peptide hepcidin is regulated by anemia, hypoxia, and inflammation. J. Clin. Invest. 110:2002;1037-1044. b
73. Nicolas G., Viatte L., Lou D.Q., Bennoun M., Beaumont C., Kahn A., Andrews N.C., Vaulont S. Constitutive hepcidin expression prevents iron overload in a mouse model of hemochromatosis. Nat. Genet. 34:2003;97-101
74. Njajou O.T., Vaessen N., Joosse M., Berghuis B., van Dongen J.W., Breuning M.H., Snijders P.J., Rutten W.P., Sandkuijl L.A., Oostra B.A., et al. A mutation in SLC11A3 is associated with autosomal dominant hemochromatosis. Nat. Genet. 28:2001;213-214
75. Oudit G.Y., Sun H., Trivieri M.G., Koch S.E., Dawood F., Ackerly C., Yazdanpanah M., Wilson G.J., Schwartz A., Liu P.P., Backx P.H. L-type Ca2+ channels provide a major pathway for iron entry into cardiomyocytes in iron-overload cardiomyopathy. Nat. Med. 9:2003;1187-1194
76. Pandolfo M. Iron metabolism and mitochondrial abnormalities in friedreich ataxia. Blood Cells Mol. Dis. 29:2002;536-547
77. Pantopoulos K., Hentze M.W. Activation of iron regulatory protein-1 by oxidative stress in vitro. Proc. Natl. Acad. Sci. USA. 95:1998;10559-10563
78. Papanikolaou G., Samuels M.E., Ludwig E.H., MacDonald M.L., Franchini P.L., Dube M.P., Andres L., MacFarlane J., Sakellaropoulos N., Politou M., et al. Mutations in HFE2 cause iron overload in chromosome 1q-linked juvenile hemochromatosis. Nat. Genet. 36:2004;77-82. Published online November 30, 2003. doi:10.1038/ng1274
79. Paraskeva E., Gray N.K., Schlager B., Wehr K., Hentze M.W. Ribosomal pausing and scanning arrest as mechanisms of translational regulation from cap-distal iron-responsive elements. Mol. Cell. Biol. 19:1999;807-816
80. Park C.H., Valore E.V., Waring A.J., Ganz T. Hepcidin, a urinary antimicrobial peptide synthesized in the liver. J. Biol. Chem. 276:2001;7806-7810
81. Parkkila S., Waheed A., Britton R.S., Bacon B.R., Zhou X.Y., Tomatsu S., Fleming R.E., Sly W.S. Association of the transferrin receptor in human placenta with HFE, the protein defective in hereditary hemochromatosis. Proc. Natl. Acad. Sci. USA. 94:1997;13198-13202
82. Perry G., Sayre L.M., Atwood C.S., Castellani R.J., Cash A.D., Rottkamp C.A., Smith M.A. The role of iron and copper in the aetiology of neurodegenerative disorders. therapeutic implications CNS Drugs. 16:2002;339-352
83. Phillips J.D., Jackson L.K., Bunting M., Franklin M.R., Thomas K.R., Levy J.E., Andrews N.C., Kushner J.P. A mouse model of familial porphyria cutanea tarda. Proc. Natl. Acad. Sci. USA. 98:2001;259-264
84. Pigeon C., Ilyin G., Courselaud B., Leroyer P., Turlin B., Brissot P., Loreal O. A new mouse liver-specific gene, encoding a protein homologous to human antimicrobial peptide hepcidin, is overexpressed during iron overload. J. Biol. Chem. 276:2001;7811-7819
85. Ponka P. Tissue-specific regulation of iron metabolism and heme synthesis. distinct control mechanisms in erythroid cells Blood. 89:1997;1-25
86. Raja K.B., Pippard M.J., Simpson R.J., Peters T.J. Relationship between erythropoiesis and the enhanced intestinal uptake of ferric iron in hypoxia in the mouse. Br. J. Haematol. 64:1986;587-593
87. Roetto A., Papanikolaou G., Politou M., Alberti F., Girelli D., Christakis J., Loukopoulos D., Camaschella C. Mutant antimicrobial peptide hepcidin is associated with severe juvenile hemochromatosis. Nat. Genet. 33:2003;21-22
88. Roy, C.N., Custodio, A.O., de Graaf, J., Schneider, S., Akpan, I., Montross, L.K., Sanchez, M., Gaudino, A., Hentze, M.W., Andrews, N.C., and Muckenthaler, M.U. (2004). An Hfe-dependent pathway mediates hyposideremia in response to lipopolysaccharide-induced inflammation in mice. Nat. Genet., in press. 10.1038/ng1350.
89. Rotig A., de Lonlay P., Chretien D., Foury F., Koenig M., Sidi D., Munnich A., Rustin P. Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia. Nat. Genet. 17:1997;215-217
90. Sturrock A., Alexander J., Lamb J., Craven C.M., Kaplan J. Characterization of a transferrin-independent uptake system for iron in HeLa cells. J. Biol. Chem. 265:1990;3139-3145
91. Taketani S., Kakimoto K., Ueta H., Masaki R., Furukawa T. Involvement of ABC7 in the biosynthesis of heme in erythroid cells. interaction of ABC7 with ferrochelatase Blood. 101:2003;3274-3280
92. Thompson K., Menzies S., Muckenthaler M., Torti F.M., Wood T., Torti S.V., Hentze M.W., Beard J., Connor J. Mouse brains deficient in H-ferritin have normal iron concentration but a protein profile of iron deficiency and increased evidence of oxidative stress. J. Neurosci. Res. 71:2003;46-63
93. Tomatsu S., Orii K.O., Fleming R.E., Holden C.C., Waheed A., Britton R.S., Gutierrez M.A., Velez-Castrillon S., Bacon B.R., Sly W.S. Contribution of the H63D mutation in HFE to murine hereditary hemochromatosis. Proc. Natl. Acad. Sci. USA. 100:2003;15788-15793. Published online December 15, 2003.
94. Torti F.M., Torti S.V. Regulation of ferritin genes and protein. Blood. 99:2002;3505-3516
95. Trenor C.C. 3rd, Campagna D.R., Sellers V.M., Andrews N.C., Fleming M.D. The molecular defect in hypotransferrinemic mice. Blood. 96:2000;1113-1118
96. Vulpe C.D., Kuo Y.M., Murphy T.L., Cowley L., Askwith C., Libina N., Gitschier J., Anderson G.J. Hephaestin, a ceruloplasmin homologue implicated in intestinal iron transport, is defective in the sla mouse. Nat. Genet. 21:1999;195-199
97. Waheed A., Grubb J.H., Zhou X.Y., Tomatsu S., Fleming R.E., Costaldi M.E., Britton R.S., Bacon B.R., Sly W.S. Regulation of transferrin-mediated iron uptake by HFE, the protein defective in hereditary hemochromatosis. Proc. Natl. Acad. Sci. USA. 99:2002;3117-3122
98. Wang J., Chen G., Muckenthaler M., Galy B., Hentze M.W., Pantopoulos K. Iron-mediated degradation of IRP2, an unexpected pathway involving a 2-oxoglutarate-dependent oxygenase activity. Mol. Cell. Biol. 24:2004;954-965
99. Weinstein D.A., Roy C.N., Fleming M.D., Loda M.F., Wolfsdorf J.I., Andrews N.C. Inappropriate expression of hepcidin is associated with iron refractory anemia. implications for the anemia of chronic disease Blood. 100:2002;3776-3781
100. Yamanaka K., Ishikawa H., Megumi Y., Tokunaga F., Kanie M., Rouault T.A., Morishima I., Minato N., Ishimori K., Iwai K. Identification of the ubiquitin-protein ligase that recognizes oxidized IRP2. Nat. Cell Biol. 5:2003;336-340
101. Yang J., Goetz D., Li J.Y., Wang W., Mori K., Setlik D., Du T., Erdjument-Bromage H., Tempst P., Strong R., Barasch J. An iron delivery pathway mediated by a lipocalin. Mol. Cell. 10:2002;1045-1056. a
102. Yang F., Liu X.B., Quinones M., Melby P.C., Ghio A., Haile D.J. Regulation of reticuloendothelial iron transporter MTP1 (Slc11a3) by inflammation. J. Biol. Chem. 277:2002;39786-39791. b
103. Zhou B., Westaway S.K., Levinson B., Johnson M.A., Gitschier J., Hayflick S.J. A novel pantothenate kinase gene (PANK2) is defective in Hallervorden-Spatz syndrome. Nat. Genet. 28:2001;345-349