Prion protein (PrP) knock-out mice show altered iron metabolism: A functional role for PrP in iron uptake and transport

PLoS ONE

Volumn 4, Issue 7, 2009, Pages

  Singh, Ajay ^a   Kong, Qingzhong ^a   Luo, Xiu ^a   Petersen, Robert B ^a   Meyerson, Howard ^a   Singh, Neena ^a  

^a CASE WESTERN RESERVE UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE; FERRITIN; IRON 59; IRON REGULATORY FACTOR; PRION PROTEIN; TRANSFERRIN; IRON;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CONTROLLED STUDY; ENDOTHELIUM CELL; ERYTHROCYTE COUNT; FERRITIN BLOOD LEVEL; HEMATOCRIT; HEMATOPOIETIC STEM CELL; HEMOGLOBIN BLOOD LEVEL; HOMEOSTASIS; IRON BINDING CAPACITY; IRON BLOOD LEVEL; IRON DEFICIENCY; IRON METABOLISM; IRON TRANSPORT; ISOTOPE LABELING; KNOCKOUT MOUSE; MOUSE; NONHUMAN; PHENOTYPE; PRION DISEASE; PURKINJE CELL; RETICULOCYTE COUNT; TRANSGENIC MOUSE; WILD TYPE; ANIMAL; BRAIN; DUODENUM; GENETICS; METABOLISM; MOUSE MUTANT; SPLEEN; TRANSPORT AT THE CELLULAR LEVEL; WESTERN BLOTTING;

ANIMALIA; MUS; MUS MUSCULUS;

ANIMALS; BIOLOGICAL TRANSPORT; BLOTTING, WESTERN; BRAIN; DUODENUM; IRON; MICE; MICE, KNOCKOUT; PHENOTYPE; PRPSC PROTEINS; SPLEEN;

EID: 67749133712     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0006115     Document Type: Article

References (30)

1. Roucou X, LeBlanc AC (2005) Cellular prion protein neuroprotective function: implications in prion diseases. J Mol Med 83: 3-11.
2. Caughey B, Baron GS (2006) Prions and their partners in crime. Nature 443: 803-810.
3. Büeler H, Fischer M, Lang Y, Bluethmann H, Lipp HP, et al. (1992) Normal development and behavior of mice lacking the neuronal cell-surface PrP protein. Nature 356: 577-582.
4. Moore RC, Mastrangelo P, Bouzamondo E, Heinrich C, Legname G, et al. (2001) Doppel-induced cerebellar degeneration in transgenic mice. Proc Natl Acad Sci USA 98: 15288-15293.
5. Weissmann C, Flechsig E (2003) PrP knock-out and PrP transgenic mice in prion research. Br Med Bull 66: 43-60.
6. Criado JR, Sánchez-Alavez M, Conti B, Giacchino JL, Wills DN, et al. (2005) Mice devoid of prion protein have cognitive deficits that are rescued by reconstitution of PrP in neurons. Neurobiol Dis 19: 255-265.
7. Weise J, Sandau R, Schwarting S, Crome O, Wrede A, et al. (2006) Deletion of cellular prion protein results in reduced Akt activation, enhanced postischemic caspase-3 activation, and exacerbation of ischemic brain injury. Stroke 37: 1296-1300.
8. Steele AD, Emsley JG, Ozdinler PH, Lindquist S, Macklis JD (2006) Prion protein (PrPc) positively regulates neural precursor proliferation during developmental and adult mammalian neurogenesis. Proc Natl Acad Sci USA 103: 3416-3421.
9. Nazor KE, Seward T, Telling GC (2007) Motor behavioral and neuropathological deficits in mice deficient for normal prion protein expression. Biochim Biophys Acta 1772: 645-653.
10. Prestori F, Rossi P, Bearzatto B, Lainé J, Necchi D, et al. (2008) Altered Neuron Excitability and Synaptic Plasticity in the Cerebellar Granular Layer of Juvenile Prion Protein Knock-Out Mice with Impaired Motor Control. J Neurosci 28: 7091-7103.
11. Zhang CC, Steele AD, Lindquist S, Lodish HF (2006) Prion protein is expressed on long-term repopulating hematopoietic stem cells and is important for their self-renewal. Proc Natl Acad Sci USA 103: 2184-2189.
12. Zivny JH, Gelderman MP, Xu F, Piper J, Holada K, et al. (2008) Reduced erythroid cell and erythropoietin production in response to acute anemia in prion protein-deficient (Prnp-/-) mice. Blood Cells Mol Dis 40: 302-307.
13. Singh A, Mohan ML, Isaac AO, Luo X, Petrak J, et al. (2009) Prion protein modulates cellular iron metabolism: Implications for prion disease pathogenesis. PLoS ONE 4: e4468.
14. Singh A, Isaac AO, Luo X, Mohan ML, Cohen ML, et al. (2009) Abnormal brain iron homeostasis in human and animal prion disorders. PLoS Pathog 5: e1000336.
15. Moos T, Morgan EH (2004) The metabolism of neuronal iron and its pathogenic role in neurological disease: review. Ann NY Acad Sci 1012: 14-26.
16. Moos T, Oates PS, Morgan EH (1998) Expression of the neuronal transferrin receptor is age dependent and susceptible to iron deficiency. J Comp Neurol 398: 420-430.
17. Chung MC (1985) A Specific iron stain for iron-binding proteins in polyacrylamide gels: application to transferrin and lactoferrin. Anal Biochem 148: 498-502.
18. Hanazawa S, Murakami Y, Hirose K, Amano S, Ohmori Y, et al. (1991) Bacteroides (Porphyromonas) gingivalis fimbriae activate mouse peritoneal macrophages and induce gene expression and production of interleukin-1. Infect Immun 59: 1972-1977.
19. Muckenthaler MU, Galy B, Hentze MW (2008) Systemic Iron Homeostasis and the Iron-Responsive Element/Iron-Regulatory Protein (IRE/IRP) Regulatory Network. Annu Rev Nutr 28: 197-213.
20. Sarnataro D, Paladino S, Campana V, Grassi J, Nitsch L, et al. (2002) PrPC is sorted to the basolateral membrane of epithelial cells independently of its association with rafts. Traffic 11: 810-821.
21. Morel E, Fouquet S, Strup-Perrot C, Thievend CP, Petit C, et al. (2008) The cellular prion protein PrP(c) is involved in the proliferation of epithelial cells and in the distribution of junction-associated proteins. PLoS ONE 3: e3000.
22. Peppriell JE, Edwards JA, Bannerman RM (1982) The Kinetics of Iron uptake by isolated intestinal cells from normal mice and mice with sex-linked anemia. Blood 60: 635-641.
23. Miura T, Sasaki S, Toyama A, Takeuchi H (2005) Copper reduction by the octapeptide repeat region of prion protein: pH dependence and implications in cellular copper uptake. Biochemistry 44: 8712-8720.
24. Hentze MW, Muckenthaler MU, Andrews NC (2004) Balancing acts: molecular control of mammalian iron metabolism. Cell 117: 285-297.
25. Pantopoulos K (2004) Iron metabolism and the IRE/IRP regulatory system: an update. Ann NY Acad Sci 1012: 1-13.
26. MacKenzie EL, Iwasaki K, Tsuji Y (2008) Intracellular Iron Transport and Storage: From Molecular Mechanisms to Health Implications. Antioxid Redox Signal 10: 997-1030.
27. Burdo JR, Connor JR (2003) Brain iron uptake and homeostatic mechanisms: an overview. Biometals 16: 63-75.
28. Fischer M, Rülicke T, Raeber A, Sailer A, Moser M, et al. (1996) Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBO J 15: 1255-1264.
29. Vyoral D, Petrák J, Hradilek A (1998) Separation of cellular iron containing compounds by electrophoresis. Biol Trace Elem Res 61: 263-275.
30. Basu S, Mohan ML, Luo X, Kundu B, Kong Q, et al. (2007) Modulation of proteinase K-resistant prion protein in cells and infectious brain homogenate by redox iron: implications for prion replication and disease pathogenesis. Mol Biol Cell 18: 3302-3312.