Review of studies that have used knockout mice to assess normal function of prion protein under immunological or pathophysiological stress

Microbiology and Immunology

Volumn 58, Issue 7, 2014, Pages 361-374

  Onodera, Takashi ^a   Sakudo, Akikazu ^b   Tsubone, Hirokazu ^a   Itohara, Shigeyoshi ^c  

^a UNIVERSITY OF TOKYO   (Japan)

^b UNIVERSITY OF THE RYUKYUS   (Japan)

^c RIKEN BRAIN SCIENCE INSTITUTE   (Japan)

Author keywords

Depression; Epilepsy; Neuroprotection; Prion protein

Indexed keywords

DEXTRAN SULFATE; GLUCOSE; ISOPROTEIN; PRION PROTEIN; PRION;

ALLERGIC ENCEPHALOMYELITIS; APOPTOSIS; ARTICLE; BACTERIAL INFECTION; BRAIN DAMAGE; BRAIN ISCHEMIA; CARDIOVIRUS INFECTION; CHRONIC DISEASE; COLITIS; DEPRESSION; DISEASE ACTIVITY; DISEASE PREDISPOSITION; DISEASE SEVERITY; ENCEPHALOMYOCARDITIS VIRUS; ENCEPHALOMYOCARDITIS VIRUS B; ENTERITIS; EPILEPSY; EPILEPTOGENESIS; EYELID REFLEX; GLUCOSE BLOOD LEVEL; HUMAN; IMMUNE RESPONSE; IMMUNOPATHOLOGY; IMPAIRED GLUCOSE TOLERANCE; IN VIVO STUDY; KNOCKOUT MOUSE; LYMPHOID TISSUE; MOUSE; NERVE CELL LESION; NEUROMUSCULAR DISEASE; NEUROPROTECTION; NONHUMAN; OXIDATIVE STRESS; PATHOPHYSIOLOGY; PREDICTION; PROTEIN DEPLETION; PROTEIN EXPRESSION; PROTEIN FUNCTION; RNA INTERFERENCE; SEIZURE; SLEEP DISORDER; SLEEP PATTERN; SMELLING DISORDER; SPATIAL LEARNING; VIRUS INFECTION; ANIMAL; DISEASE MODEL; EXPERIMENTAL AUTOIMMUNE ENCEPHALOMYELITIS; GENE INACTIVATION; GENETICS; IMMUNITY; IMMUNOLOGY; METABOLISM; PHYSIOLOGICAL STRESS; PRION;

ANIMALS; BACTERIAL INFECTIONS; BRAIN ISCHEMIA; CARDIOVIRUS INFECTIONS; COLITIS; DEPRESSION; DISEASE MODELS, ANIMAL; DISEASE SUSCEPTIBILITY; ENCEPHALOMYELITIS, AUTOIMMUNE, EXPERIMENTAL; EPILEPSY; GENE KNOCKOUT TECHNIQUES; HUMANS; IMMUNITY; MICE; MICE, KNOCKOUT; PRIONS; RNA INTERFERENCE; STRESS, PHYSIOLOGICAL;

EID: 84903885282     PISSN: 03855600     EISSN: 13480421     Source Type: Journal    
DOI: 10.1111/1348-0421.12162     Document Type: Article

References (126)

1. Onodera T., Sakudo A., Wu G., Saeki K. ( 2006) Bovine spongiform encephalopathy in Japan: history and recent studies on oxidative stress in prion diseases. Microbiol Immunol 50: 565-78.
2. Sakudo A., Lee D.C., Saeki K., Nakamura Y., Inoue K., Matsumoto Y., Itohara S., Onodera T. ( 2003) Impairment of superoxide dismutase activation by N-terminally truncated prion protein (PrP) in PrP-deficient neuronal cell line. Biochem Biophys Res Commun 308: 660-7.
3. Sakudo A., Nakamura I., Ikuta K., Onodera T. ( 2007) Recent developments in prion disease research: diagnostic tools and in vitro cell culture models. J Vet Med Sci 69: 329-37.
4. Brown H.R., Goller N.L., Rudelli R.D., Merz G.S., Wolfe G.C., Wisniewski H.M., Robakis N.K. ( 1990) The mRNA encoding the scrapie agent protein is present in a variety of non-neuronal cells. Acta Neuropathol (Berl) 80: 1-6.
5. Cashman N.R., Loertscher R., Nalbantoglu J., Shaw I., Kascsak R.J., Bolton D.C., Bendheim P.E. ( 1990) Cellular isoform of the scrapie agent protein participates in lymphocyte activation. Cell 61: 185-92.
6. Manson J., West J.D., Thomson V., McBride P., Kaufman M.H., Hope J. ( 1992) The prion protein gene: a role in mouse embryogenesis. Development 115: 117-22.
7. Moser M., Colello R.J., Pott U., Oesch B. ( 1995) Developmental expression of the prion protein gene in glial cells. Neuron 14: 509-17.
8. Sakudo A., Onodera T., Ikuta K. ( 2007) Prion protein gene-deficient cell lines: powerful tools for prion biology. Microbiol Immunol 51: 1-13.
9. Pan T., Wong B.S., Liu T., Li R., Petersen R.B., Sy M.S. ( 2002) Cell-surface prion protein interacts with glycosaminoglycans. Biochem J 368: 81-90.
10. Graner E., Mercadante A.F., Zanata S.M., Forlenza O.V., Cabral A.L., Veiga S.S., Juliano M.A., Roesler R., Walz R., Minetti A., Izquierdo I., Martins V.R., Brentani R.R. ( 2000) Cellular prion protein binds laminin and mediates neuritogenesis. Brain Res Mol Brain Res 76: 85-92.
11. Schimitt-Ulms G., Legname G., Baidwin M.A., Ball H.L., Brandon N., Bosque P.J., Crossin K.L., Edelman G.M., DeArmond S.J., Cohen F.E., Prusiner S.B. ( 2001) Binding of neural adhesion molecules (N-CAMs) to the cellular prion protein. J Mol Biol 314: 1209-25.
12. C directly interacts with proteins involved in signaling pathways. J Biol Chem 276: 44604-12.
13. Zanata S.M., Lopes M.H., Mercadante A.F., Hajj G.N., Chiarini L.B., Nomizo R., Freitas A.R., Cabral A.L., Lee K.S., Juliano M.A., de Oliveira E., Jachieri S.G., Burlingame A., Huang L., Linden R., Brentani R.R., Martins V.R. ( 2002) Stress-inducible protein 1 is a cell surface ligand for cellular protein that triggers neuroprotection. EMBO J 21: 3307-16.
14. Kurschner C., Morgan J.I. ( 1996) Analysis of interaction sites in homo- and heteromeric complex containing Bcl-2 family members and the cellular prion protein. Brain Res Mol Brain Res 37: 249-58.
15. Kuwahara C., Takeuchi A.M., Nishimura T., Haraguchi K., Kubosaki A., Matsumoto Y., Saeki K., Matsumoto Y., Yokoyama T., Itohara S., Onodera T. ( 1999) Prions prevent neuronal cell-line death. Nature 400: 225-6.
16. Brown D.R., Nicholas R.S., Canevari L. ( 2002) Lack of prion protein expression results in a neuronal phenotype sensitive to stress. J Neurosci Res 67: 211-24.
17. Wong B.S., Liu T., Li R., Pan T., Petersen R.B., Smith M.A., Gambetti P., Perry G., Manson J.C., Brown D.R., Sy M.S. ( 2001) Increased levels of oxidative stress markers detected in the brains of mice devoid of prion protein. J Neurochem 76: 565-72.
18. Rachidi W., Vilette D., Guiraud P., Ariotto M., Riondel J., Laude H., Lehmann S., Favier A. ( 2003) Expression of prion protein increases cellular copper binding and antioxidant enzyme activities but not copper delivery. J Biol Chem 278: 9064-72.
19. Walz R., Amaral O.B., Rockenbach I.C., Roesler R., Izquierdo I., Cavalheiro E.A., Martins V.R., Brentani R.R. ( 1999) Increased sensitivity to seizure in mice lacking cellular prion protein. Epilepsia 40: 1679-82.
20. Weiss J., Sandau R., Schwarting S., Crome O., Wrede A., Schulz-Schaeffer W., Zerr I., Bahr M. ( 2006) Deletion of cellular prion protein results in reduced Akt activation, enhanced postischemic caspase-3 activation, and exacerbation of ischemic brain injury. Stroke 37: 1296-300.
21. C by adenovirus-mediated gene targeting reduces ischemic injury in a stroke rat model. J Neurosci 25: 8967-77.
22. Tsutsui S., Nahn J.H., Johnson T.A., Ali Z., Jirik F.R. ( 2008) Absence of cellular prion protein exacerbates and prolongs neuroinflammation in experimental autoimmune encephalomyelitis. Am J Pathol 173: 1029-41.
23. Cashmann N.R., Loertscher R., Nalbantoglu J., Shaw I., Kascsak R.J., Bolton D.C., Bendheim P.E. ( 1990) Cellular isoform of the scrapie agent protein participates in lymphocyte activation. Cell 61: 185-92.
24. Paar C., Wurm S., Pfarr W., Sonnleitner A., Wechselberger C. ( 2007) Prion protein resides in membrane microclusters of the immunological synapses during lymphocyte activation. Eur J Cell Biol 86: 253-64.
25. Issacs D.J., Jackson G.S., Altmann D.M. ( 2006) The role of cellular prion protein in the immune system. Clin Exp Immunol 146: 1-8.
26. Ballerini C., Gourdain P., Bachy V., Blanchard N., Levavasseur E., Gregoire S., Fontes P., Aucouturier P., Hivroz C., Carnaud C. ( 2006) Functional implication of cellular prion protein in antigen-driven interactions between T cells and dendritic cells. J Immunol 176: 7254-62.
27. Bar-Or A., Oliveira E.M., Anderson D.E., Hafler D.A. ( 1999) Molecular pathogenesis of multiple sclerosis. J Neuroimmunol 100: 252-9.
28. Steinman L. ( 2007) A brief history of T(H) 17, the first major revision in the T(H) 1/T(H) 2 hypothesis of T cell-mediated tissue damage. Nat Med 13: 139-45.
29. Lock C., Hermans G., Pedotti R., Brendolan A., Schadt E., Garren H., Langer-Gould A., Strober S., Cannella B., Allard J., Klonowski P., Austin A., Lad N., Kaminski N., Galli S.J., Oksenberg J.R., Raine C.S., Heller R., Steinman L. ( 2002) Gene-microarray analysis of multiple sclerosis lesions yields new targets validated in autoimmune encephalomyelitis. Nat Med 8: 500-8.
30. Marciano P.G., Brettschneider J., Manduchi E., Davis J.E., Eastman S., Raghupathi R., Saatman K.E., Speed T.P., Stoeckert C.J., Jr., Eberwine J.H., Mclntosh T.K. ( 2004) Neuron-specific mRNA complexity responses during hippocampal apoptosis after traumatic brain injury. J Neurosci 24: 2866-76.
31. Singh A., Kong Q., Luo X., Petersen R.B., Meyerson H., Singh N. ( 2009) Prion protein (PrP) knock-out mice show altered iron metabolism: a functional role for PrP in iron uptake and transport. PLoS ONE 4: e6115.
32. Brown D.R. ( 2009) Brain protein that mind metals: a neurodegenerative perspective. Dalton Trans 21: 4069-76.
33. Nishimura T., Sakudo A., Hashiyama Y., Yachi A., Saeki K., Matsumoto Y., Ogawa M., Sakaguchi S., Itohara S., Onodera T. ( 2007) Serum withdrawal-induced apoptosis in ZrchI prion protein (PrP) gene-deficient neuronal cell line is suppressed by PrP, independent of Doppel. Microbiol Immunol 51: 457-66.
34. Watt N.T., Taylor D.R., Gillott A., Thomas D.A., Perera W.S., Hooper N.M. ( 2005) Reactive oxygen species-mediated beta-cleavage of the prion protein in the cellular response to oxidative stress. J Biol Chem 380: 35914-21.
35. Martin G.R., Keenan C.M., Sharkey K.A., Jirik F.R. ( 2011) Endogenous prion protein attenuates experimentally induced colitis. Am J Pathol 179: 2290-301.
36. Kim C.K., Hirose Y., Sakudo A., Takeyama N., Kang C.B., Taniuchi Y., Matsumoto Y., Itohara S., Sakaguchi S., Onodera T. ( 2007) Reduced response of splenocytes after mitogen-stimulation in the prion protein (PrP) gene-deficient mouse: PrPLP/Doppel production and cerebral degeneration. Biochem Biophys Res Commun 358: 469-74.
37. Ingram R.J., Issacs J.D., Kaur G., Lowther D.E., Reynolds C.J., Boyton R.J., Collinge J., Jackson G.S., Altmann D.M. ( 2009) A role of cellular prion protein in programming T-cell cytokine responses in disease. FESB J 23: 1672-84.
38. Krebs B., Dorner-Ciossek C., Schmalzbauer R., Vassallo N., Herms J., Kretzschmar H.A. ( 2006) Prion protein induced signaling cascades in monocytes. Biochem Biophys Res Commun 340: 13-22.
39. De Almeida C.J., Chiarini L.B., da Silva J.P., Silva E.P.M., Martins M.A., Linden R. ( 2005) The cellular prion protein modulates phagocytosis and inflammatory response. J Leukoc Biol 77: 238-46.
40. Hu W., Nessler S., Hemmer B., Eager T.N., Kane L.P., Leiliveld S.R., Muller-Schiffmann A., Gocke A.R., Lovett-Racke A., Ben L.H., Hussain R.Z., Breil A., Elliott J.L., Puttaparthi K., Cravens P.D., Singh M.P., Petsch B., Stitz L., Racke M.K., Kroth C., Stuve O. ( 2010) Pharmacological prion protein silencing accelerates central nervous system autoimmune disease via T cell receptor signaling. Brain 133: 375-88.
41. Estaff-Leung N., Mabarrack N., Barbour A., Cummins A., Barrys S. ( 2010) Foxp3+ regulatory T cells: Th17 effecter cells, and cytokine environment in inflammatory bowel disease. J Clin Immunol 30: 80-9.
42. Thackray A.M., McKenzie A.N., Klein M.A., Lauder A., Bujdoso R. ( 2004) Accelerated prion disease in the absence of interleukin-10. J Virol 78: 13697-707.
43. Stoeck K., Bodemer M., Ciesielczyk B., Meissner B., Batil M., Heinemann U., Zerr I. ( 2005) Interleukin 4 and interleukin 10 levels are elevated in the cerebrospinal fluid of patents with Creutzfeldt-Jakob diseases. Arch Neurol 62: 1591-4.
44. Pasupuleti M., Roupe M., Rydengard V., Surewicz K., Surewicz W.K., Chalupka A., Malmsten M., Sorensen O.E., Schmidtchen A. ( 2009) Antimicrobial activity of human prion protein is mediated by its N-terminal region. PLoS ONE 4: e7358.
45. Hanock R.E., Diamond G. ( 2000) The role of cationic antimicrobial peptides in innate host defenses. Trends Microbiol 8: 402-10.
46. Xavier R.J., Podolsky D.K. ( 2007) Unraveling the pathogenesis of inflammatory bowel disease. Nature 448: 427-34.
47. Li Y., de Haar C., Chen M., Deuring J., Gerritis M.M., Smits R., Xia B., Kuipers E.J., van der Woude C.J. ( 2010) Disease-related expression of the IL6/STAT3/SOCS3 signaling pathway in ulcerative colitis and ulcerative colitis-related carcinogenesis. Gut 59: 227-35.
48. Bishop M.T., Pennington C., Heath C.A., Will R.G., Knight R.S. ( 2009) PRNP variant in UK sporadic and variant Creutzfeldt-Jakob disease high-light genetic risk factors and a novel non-synonymous polymorphism. BMC Med Genet 10: 146.
49. Sudich A., Frigg R., Kilic U., Oesch B., Raeber A., Bassetti C.L., Hermann D.M. ( 2005) Aggravation of ischemic brain injury by prion protein deficiency: role of FER-1/-2 and STAT-1. Neurobiol Dis 20: 442-9.
50. Watanabe T., Yasutaka Y., Nishioku T., Kusakabe S., Futagami K., Yamaguchi A., Kataoka Y. ( 2011) Involvement of the cellular prion protein in the migration of brain microvascular endothelial cells. Neurosci Lett 496: 121-4.
51. Mitterrgger G., Vosko M., Krebs B, Xiang W., Kohlmannsperger V., Nolting S., Hamann G.F., Kretzschmar H.A. ( 2007) The role of octarepeat region in neuroprotective function of the cellular prion protein. Brain Pathol 17: 174-83.
52. Beraldo F.H., Arantes C.P., Santos T.G., Queiroz N.G., Young K., Rylett R.J., Markus R.P., Prado M.A., Martins V.R. ( 2010) Role of alpha7 nicotonic acetylcholine receptor in calcium signaling induced by prion protein interaction with stress-inducible protein 1. J Biol Chem 285: 36542-50.
53. Schneider B., Mutel V., Pietri M., Ernoval M., Mouillet-Richard S, Kellermann O ( 2003) NAPDH oxidase and extracellular regulated kinases 1/2 are targets of prion protein signaling in neuronal and nonneuronal cells. Proc Natl Acad Sci USA 100: 13326-31.
54. Pradines E., Loubet D., Schneider B., Launay J.M., Kellermann O., Mouillet-Richard S. ( 2008) CREB-dependent gene regulation by prion protein: impact on MMP-9 and beta-dystroglycan. Cell Signal 20: 2050-8.
55. Pradines E., Loubet D., Mouillet-Richard S., Manivet P., Launay J.M., Kellermann O., Schneider B. ( 2009) Cellular prion protein coupling to TACE-dependent TNF-alpha shedding controls neurotransmitter catabolism in neuronal cells. J Neurochem 110: 912-23.
56. Linden R., Martins V.R., Prado M.A., Cammarota M., Izquierdo I., Brentani R.R. ( 2008) Physiology of the prion protein. Physiol Rev 88: 673-728.
57. Moreno J.A., Radford H., Peretti D., Steinert J.R., Verity N., Martin M.G., Halliday M., Morgan J., Dinsdale D., Ortori C.A., Barrett D.A., Tsaytler P., Bertolotti A., Willis A.E., Bushell M., Mallucci G.R. ( 2012) Sustained translational repression by elF2α-P mediates prion neurodegeneration. Nature 485: 507-11.
58. Ashe K.H., Aguzzi A. ( 2013) Prions, prionoids and pathogenic proteins in Alzheimer disease. Prion 7: 55-9.
59. Kristiansen M., Deriziotis P., Dimcheff D.E., Jackson G.S., Ovaa H., Naumann H., Clarke A.R., van Leeuwen F.W., Menendez-Benito V., Dantuma N.P., Portis J.L., Collinge J., Tabriz S.J. ( 2007) Disease-associated prion protein oligomers inhibit the 26S proteasome. Mol Cell 26: 175-88.
60. Milhavet O., Lehmann S. ( 2002) Oxidative stress and the prion protein in transmissible spongiform encephalopathies. Brain Res Brain Res Rev 38: 328-39.
61. C signaling pathways by prion peptide 106-126 causes oxidative injury of bioaminergic neuronal cells. J Biol Chem 281: 28470-9.
62. Heiseke A., Agrib Y., Schatzl H.M. ( 2010) Autophagy, prion infection and their mutual interactions. Curr Issues Mol Biol 12: 87-98.
63. Hoshino S., Inoue K., Yokoyama T., Kobayashi S., Asakura T., Teramoto A., Itohara S. ( 2003) Prions prevent brain damage after experimental brain injury: a preliminary report. Acta Neurochir Suppl 86: 297-9.
64. Matins V.R., Linden R., Prado M.A.M., Walz R., Sakamoto A.C., Izquierdo I., Brentani R.R. ( 2002) Cellular prion protein: on the road for functions. FEBS Lett 512: 25-8.
65. Dal Pizzol F., Klamt F., Vianna M., Schoder N., Quevedo J., Moreira J.C., Walz R. ( 2000) Lipid peroxidation in hippocampus early and late after status epilepticus induced by pilocarpine or kainic acid in Wistar rats. Neurosci Lett 291: 179-82.
66. Klamt F., Dal-Pizzol F., Conte da Frota, Walz M.L., Andrades R., da Silva M.E., Brentani E.G., Izquierdo R.R., Fonseca I., Moreira J.C. ( 2001) Imbalance of antioxidant defense in mice lacking cellular prion protein. Free Rad Biol Med 30: 1137-44.
67. Pereira G.S., Walz R., Bonan C.D., Battastini A.M., Izquierdo I., Martins V.R., Brentani R.R., Sarkis J.J. ( 2001) Changes in cortical and hippocampal ectonucleotidase activities in mice lacking cellular prion protein. Neurosci Lett 301: 72-4.
68. Samaia H.B., Mari J.J., Vallada H.P., Moura R.P., Simpson A.T.G., Brentani R.R. ( 1997) A prion-linked psychiatric disorder. Nature 390: 241.
69. Fournier J.G., Escaig-Haye F., Grigoriev V. ( 2000) Ultrastructural localization of prion proteins: physiological and pathological implications. Microsc Res Tec 50: 324-9.
70. Fournier J.G. ( 2008) Cellular prion protein electron microscopy: attempts/limits and clues to a synaptic trait. Implication in neurodegeneration process. Cell Tissue Res 332: 1-11.
71. C against-kainate-induced epileptic seizures and cell death depends on the modulation of JNK3 activation by GluR6/7-PSD-95 binding. Mol Biol Cell 22: 3041-54.
72. Steele A.D. ( 2008) All quiet on the neuronal front: NMDA receptor inhibition by prion protein. J Cell Biol 181: 407-9.
73. Ratte S., Vreugdenhil M., Boult J.K., Patel A., Asante E.A., Collinge J., Jefferys J.G. ( 2011) Threshold of epileptiform activity is elevated in prion knockout mice. Neuroscience 179: 56-61.
74. Gadotti V.M., Bonfield S.P., Zamponi G.W. ( 2012) Depressive-like behavior of mice lacking cellular prion protein. Behav Brain Res 227: 319-23.
75. Gadotti V.M., Zamponi G.W. ( 2011) Cellular prion protein protects from inflammatory and neuropathic pain. Mol Pain 7: 59.
76. Stys P.K., You H., Zamponi G.W. ( 2012) Copper-dependent regulation of NMDA receptors by cellular prion protein: implications for neurodegenerative disorders. J Physiol 590: 1357-68.
77. Nasu-Nishimura Y., Taniuchi Y., Nishimura T., Sakudo A., Nakajima K., Ano Y., Sugiura K., Sakaguchi S., Itohara S., Onodera T. ( 2008) Cellular prion protein prevents brain damage after encephalomyocarditis virus (EMCV) infection in mice. Arch Virol 153: 1007-12.
78. Keshet G.I., Ovadia H., Taraboulos A., Gabizon R. ( 1999) Scrapie-infected mice and PrP knockout mice share abnormal localization and activity of neuronal nitric oxide synthase. J Neurochem 72: 1224-31.
79. Kitamura Y., Yanagisawa D., Inden M., Takata K., Tsuchiya D., Kawasaki T., Taniguchi T., Shimohama S. ( 2005) Recovery of focal brain ischemia-induced behavior dysfunction by intracerebroventricular injection of microglia. J Pharmacol Sci 97: 289-93.
80. Roucou X., LeBlanc A.C. ( 2005) Cellular prion protein neuroprotective function: implications in prion diseases. J Mol Med 83: 3-11.
81. Sakudo A., Onodera T., Suganuma Y., Kobayashi T., Saeki K., Ikuta K. ( 2006) Recent advances in clarifying prion protein functions using knockout mice and derived cell lines. Mini Rev Med Chem 6: 589-601.
82. Dal-Pizzol F., Klamt F., Vianna M.M., Schroder N., Quevedo J., Benfato M.S., Moreira J.C., Walz R. ( 2000) Lipid peroxidation in hippocampus early and late after status epilepticus induced by pilocarpine or kainic acid in Wistar rats. Neurosci Lett 291: 179-82.
83. Hafiz F.B., Brown D.R. ( 2000) A model for the mechanism of astrogliosis in prion disease. Mol Cell Neurosci 16: 221-32.
84. Wong B.S., Liu T., Li R., Pan T., Petersen R.B., Smith M.A., Gambetti P., Perry G., Manson J.C., Brown D.R., Sy M.S. ( 2001) Increased levels of oxidative stress markers detected in the brains of mice devoid of prion proteins. J Neurochem 76: 565-72.
85. Sriskandan S., Unnikrishnan M., Krausz T., Dewchand H., Van Noorden S., Cohen J., Altmann D.M. ( 2001) Enhanced susceptibility to superantigen-associated streptococcal sepsis in human leukocyte antigen-DQ transgenic mice. J Infect Dis 184: 166-73.
86. Faulkner L., Cooper A., Fantino C., Altmann D.M., Sriskandan S. ( 2005) The mechanism of superantigen-mediated toxic shock: not a simple Th1 cytokine storm. J Immunol 175: 6870-7.
87. Sriskandan S., Altmann D.M. ( 2008) The immunology of sepsis. J Pathol 214: 211-23.
88. Mariante R.M., Nobrega A., Martins R.A.P., Areal R.B., Bellio M., Linden R. ( 2012) Neuroimmunoendocrine regulation of the prion protein in neurtophils. J Biol Chem 287: 35506-15.
89. Silva M.T. ( 2010) When two is better than one. Macrophage and neutrophils work in concert in innate immunity as complementary and cooperative partner of a myeloid phagocyte system. J Leukocyte Biol 87: 93-106.
90. Strom A., Wang G.S., Reimer R., Scott F.W. ( 2007) Pronounced cytosolic aggregation of cellular prion protein in pancreatic beta-cells in response to hyperglycemia. Lab Invest 87: 139-49.
91. Bailey J.D., Berardinelli J.G., Rocke T.E., Bessen R.A. ( 2008) Prominent pancreatic endocrinopathy and altered control of food intake disrupt energy homeostasis in prion diseases. J Endocrinol 197: 251-63.
92. Carp R.I., Kim Y.S., Callahan S.M. ( 1989) Scrapie-induced alterations in glucose tolerance in mice. J Gen Virol 70: 827-35.
93. Carp R.I., Kim Y.S., Callahan S.M. ( 1990) Pancreatic lesions and hypoglycemia-hyperinsulinemia in scrapie-infected hamsters. J Infect Dis 161: 462-6.
94. Ye X., Carp R.I., Yu Y., Kozielski R., Kozlowski P. ( 1994) Hyperplasia and hypertrophy of B cells in the islets of Langerhans in hamsters infected with the 139H strains of scrapie. J Comp Pathol 110: 169-83.
95. Ye X., Carp R.I., Kascsak R.J. ( 1994) Histopathological changes in the islets of Langerhans in scrapie 139H-affected hamsters. J Comp Pathol 110: 153-67.
96. Ye X., Carp R.I. ( 1996) Margination and diapedesis of inflammatory cells in the islets of Langerhans in hamsters infected with the 139H strain of scrapie. J Comp Pathol 114: 149-63.
97. Ye X., Carp R.I. ( 1996) Histopathological changes in islets of Langerhans in hamsters infected with 139H strain of scrapie: semi-thin section study. Histol Histopathol 11: 161-70.
98. Strom A., Wang G.S., Scott F.W. ( 2011) Impaired glucose tolerance in mice lacking cellular prion protein. Pancreas 40: 229-32.
99. Macotela Y., Boucher J., Tran T.T., Kahn C.R. ( 2009) Sex and depot differences in adipocyte insulin sensitivity and glucose metabolism. Diabetes 58: 803-12.
100. Kim C.K., Sakudo A., Taniuchi Y., Shigematsu K., Kang C.B., Saeki K., Matsumoto Y., Sakaguchi S., Itohara S., Onodera T. ( 2007) Late-onset olfactory deficits and mitral cell loss in mice lacking prion protein with ectopic expression of Doppel. Int J Mol Med 20: 169-76.
101. Le Pichon C.E., Valley M.T., Polymenidou M., Chesler A.T., Sagdullaev B.T., Aguzzi A., Firestein S. ( 2009) Olfactory behavior and physiology are disrupted in prion protein knockout mice. Nat Neurosci 12: 60-9.
102. Nico P.B., Lobao-Soares B., Landemberger M.C., Marques W., Jr, Tasca C.I., de Mello C.F., Walz R., Carlotti C.G., Jr, Brentani R.R., Sakamoto A.C., Bianchin M.M. ( 2005) Impaired exercise capacity, but unaltered mitochondrial respiration in skeletal or cardiac muscle of mice lacking cellular prion protein. Neurosci Lett 388: 21-6.
103. Nico P.B., de-Paris F., Viande E.R., Amaral O.B., Rockenbach I., Soares B.L., Guarnieri R., Wichert-Ana L., Calvo F., Walz R., Izquierdo I., Sakamoto A.C., Brentani R., Martins V.R., Bianchin M.M. ( 2005) Altered behavioral response to acute stress in mice lacking cellular prion protein. Behav Brain Res 162: 173-81.
104. Coitinho A.S., Dietrich M.O., Hoffmann A., Dall'lgna O.P., Souza D.O., Martins V.R., Brenatni R.R., Izquierdo L., Lara D.R. ( 2002) Decreased hyperlocomotion induced by MK-801, but not amphetamine and caffeine in mice lacking cellular prion protein (PrP(C)). Brain Res Mol Brain Res 107: 190-4.
105. Coitinho A.S., Roesler R., Martins V.R., Brentani R.R., Izquierdo I. ( 2003) Cellular prion protein ablation impairs behavior as a function of age. NeuroReport 14: 1375-9.
106. Prusiner S.B., Scott M.R., DeArmond S.J., Cohen F.E. ( 1998) Prion protein biology. Cell 93: 337-48.
107. Izquierdo I., Medina J.H., Vianna M.R., Izquierdo L.A., Barros D.M. ( 1999) Separate mechanisms for short- and Ling-term memory. Behav Brain Res 103: 1-11.
108. Criado J.R., Sanchez-Alavez M., Conti B., Giacchino J.L., Wills D.N., Henriksen S.J., Race R., Manson J.C., Chesebro B., Oldstone M.B. ( 2005) Mice devoid of prion protein have cognitive deficits that are rescued by reconstruction of PrP in neurons. Neurobiol Dis 19: 255-65.
109. Barnes C.A. ( 1979) Memory deficits associated with senescence: a neurophysiological and behavioral study in rat. J Comp Physiol Psychol 93: 74-104.
110. Sanchez-Alavez M., Conti B., Moroncini G., Criado J.R. ( 2007) Contributions of neuronal prion protein on sleep recovery and stress response following sleep deprivation. Brain Res 1158: 71-80.
111. Kim J.J., Thompson R.F. ( 1997) Cerebellar circuits and synaptic mechanism involved in classical eyeblink conditioning. Trends Neurosci 20: 177-81.
112. Thompson R.F., Kim J.J. ( 1996) Memory systems in the brain and localization of a memory. Proc Natl Acad Sci USA 93: 13438-44.
113. Thompson R.F., Krupa D.J. ( 1994) Organization of a memory trace in the mammalian brain. Annu Rev Neurosci 17: 519-49.
114. Attwell P.J., Cooke S.F., Yeo C.H. ( 2002) Cerebellar function in consolidation of a motor memory. Neuron 34: 1011-20.
115. Bracha V. ( 2004) Role of the cerebellum in eyeblink conditioning. Prog Brain Res 143: 331-9.
116. Krupa D.J., Thompson J.K., Thompson R.F. ( 1993) Localization of a memory trace in the mammalian brain. Science 1260: 989-91.
117. Kishimoto Y., Hirono M., Atarashi R., Sakaguchi S., Yoshioka T., Katamine S., Kirino Y. ( 2013) Age-dependent impairment of eyeblink conditioning in prion protein-deficient mice. PLoS ONE 8: e60627.
118. Sailer A., Bueler H., Fischer M., Aguzzi A., Weissmann C. ( 1994) No propagation of prions in mice devoid of PrP. Cell 77: 967-8.
119. White M.D., Farmer M., Mirabile I., Brander S., Collinge J., Mallucci G.R. ( 2008) Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease. Proc Natl Acad Sci USA 105: 10238-43.
120. White M.D., Mallucci G.R. ( 2009) RNAi for the treatment of prion disease: a window for intervention in neurodegeneration?. CNS Neurol Disord Drug Targets 8: 342-52.
121. Tilly G., Chapuis J., Vilette D., Laude H., Vilotte JL. ( 2003) Efficient and specific down-regulation of prion protein expression by RNAi. Biochem Biophys Res Commun 305: 548-51.
122. Pfeifer A., Eigenbrod S., Al-Khadra S., Fofmann A., Mitteregger G, Moser M., Bertsch U., Kretzschmar H. ( 2006) Lentivector-mediated RNAi efficiently suppresses prion protein and prolongs survival of scrapie-infected mice. J Clin Invest 116: 3204-10.
123. Manson J.C., Clarke A.R., Hooper M.L., Aitchison L., NcConnell I., Hope J. ( 1994) 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal. Mol Neurobiol 8: 121-7.
124. Fischer M., Rulicke T., Raeber A., Sailer A., Moser M., Oesch B., Brandner S., Aguzzi A., Weissmann C. ( 1996) Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBO J 15: 1255-64.
125. Lauren J., Gimbel D.A., Nygaard H.B., Gilbert J.W., Strittmatter S.M. ( 2009) Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers. Nature 457: 1128-32.
126. Nah J., Pyo J.O., Jung S., Yoo S.M., Kam T.I., Chang J., Han J., An S.S.A., Onodera T., Jung Y.K. ( 2013) BECN1/Beclin1 is recruited into lipid rafts by prion to activate autophagy in response to amyloid β42. Autophagy 9: 2009-22.