Correction to: Fatal Prion Disease in a Mouse Model of Genetic E200K Creutzfeldt-Jakob Disease (PLoS Pathogens, (2011), 7, 11, (e1002350), 10.1371/journal.ppat.1002350);Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob disease

PLoS Pathogens

Volumn 7, Issue 11, 2011, Pages

  Friedman Levi, Yael ^a   Meiner, Zeev ^a   Canello, Tamar ^a   Frid, Kati ^a   Kovacs, Gabor G ^b   Budka, Herbert ^b   Avrahami, Dana ^a   Gabizon, Ruth ^a  

^a Agnes Ginges Center for Human Neurogenetics   (Israel)

^b MEDICAL UNIVERSITY OF VIENNA   (Austria)

Author keywords

[No Author keywords available]

Indexed keywords

BRAIN EXTRACT; GLUTAMIC ACID; LYSINE; PRION PROTEIN; PROTEINASE K; HYBRID PROTEIN;

AMINO ACID SUBSTITUTION; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BRAIN TISSUE; CODON; CONTROLLED STUDY; CREUTZFELDT JAKOB DISEASE; DEATH; GENE MUTATION; HETEROZYGOTE; HISTOPATHOLOGY; HUMAN; IMMUNOBLOTTING; INOCULATION; JEW; LIBYAN ARAB JAMAHIRIYA; NEUROLOGIC DISEASE; NONHUMAN; NULL ALLELE; PRION DISEASE; PROPHYLAXIS; SPINAL CORD; TRANSGENIC MOUSE; WILD TYPE; ANIMAL; BRAIN; DISEASE COURSE; DISEASE MODEL; DISEASE TRANSMISSION; GENETICS; METABOLISM; MOUSE; PATHOLOGY; PRION;

ANIMALIA; MUS;

ANIMALS; BRAIN; CREUTZFELDT-JAKOB SYNDROME; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; HUMANS; MICE; MICE, TRANSGENIC; PRIONS; RECOMBINANT FUSION PROTEINS;

EID: 81755183039     PISSN: 15537366     EISSN: 15537374     Source Type: Journal    
DOI: 10.1371/journal.ppat.1006294     Document Type: Erratum

References (47)

1. Kovacs GG, Puopolo M, Ladogana A, Pocchiari M, Budka H, et al. (2005) Genetic prion disease: the EUROCJD experience. Hum Genet 118: 166-174.
2. Hsiao K, Prusiner SB, (1990) Inherited human prion diseases. Neurology 40: 1820-1827.
3. Hsiao K, Meiner Z, Kahana E, Cass C, Kahana I, et al. (1991) Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. N Engl J Med 324: 1091-1097.
4. Korczyn AD, Chapman J, Goldfarb LG, Brown P, Gajdusek DC, (1991) A mutation in the prion protein gene in Creutzfeldt-Jakob disease in Jewish patients of Libyan, Greek, and Tunisian origin. Ann N Y Acad Sci 640: 171-176.
5. Lee HS, Sambuughin N, Cervenakova L, Chapman J, Pocchiari M, et al. (1999) Ancestral origins and worldwide distribution of the PRNP 200K mutation causing familial Creutzfeldt-Jakob disease. Am J Hum Genet 64: 1063-1070.
6. Stewart LA, Rydzewska LH, Keogh GF, Knight RS, (2008) Systematic review of therapeutic interventions in human prion disease. Neurology 70: 1272-1281.
7. Geschwind MD, (2009) Clinical trials for prion disease: difficult challenges, but hope for the future. Lancet Neurol 8: 304-306.
8. Otto M, Cepek L, Ratzka P, Doehlinger S, Boekhoff I, et al. (2004) Efficacy of flupirtine on cognitive function in patients with CJD: A double-blind study. Neurology 62: 714-718.
9. Brown P, Gibbs CJ Jr, Rodgers-Johnson P, Asher DM, Sulima MP, et al. (1994) Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 35: 513-529.
10. Tateishi J, Kitamoto T, (1995) Inherited prion diseases and transmission to rodents. Brain Pathol 5: 53-59.
11. Groschup MH, Buschmann A, (2008) Rodent models for prion diseases. Vet Res 39: 32.
12. Solomon IH, Schepker JA, Harris DA, (2010) Prion neurotoxicity: insights from prion protein mutants. Curr Issues Mol Biol 12: 51-61.
13. Hsiao KK, Scott M, Foster D, Groth DF, DeArmond SJ, et al. (1990) Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science 250: 1587-1590.
14. Hsiao KK, Groth D, Scott M, Yang SL, Serban H, et al. (1994) Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proc Natl Acad Sci U S A 91: 9126-9130.
15. Telling GC, Scott M, Hsiao KK, Foster D, Yang SL, et al. (1994) Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad Sci U S A 91: 9936-9940.
16. Chiesa R, Piccardo P, Ghetti B, Harris DA, (1998) Neurological illness in transgenic mice expressing a prion protein with an insertional mutation. Neuron 21: 1339-1351.
17. Yang W, Cook J, Rassbach B, Lemus A, DeArmond SJ, et al. (2009) A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP. J Neurosci 29: 10072-10080.
18. Dossena S, Imeri L, Mangieri M, Garofoli A, Ferrari L, et al. (2008) Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model. Neuron 60: 598-609.
19. Jackson WS, Borkowski AW, Faas H, Steele AD, King OD, et al. (2009) Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice. Neuron 63: 438-450.
20. Asante EA, Gowland I, Grimshaw A, Linehan JM, Smidak M, et al. (2009) Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins. J Gen Virol 90: 546-558.
21. Telling GC, Haga T, Torchia M, Tremblay P, DeArmond SJ, et al. (1996) Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes Dev 10: 1736-1750.
22. Scott MR, Kohler R, Foster D, Prusiner SB, (1992) Chimeric prion protein expression in cultured cells and transgenic mice. Protein Sci 1: 986-997.
23. Kovacs GG, Budka H, (2009) Molecular pathology of human prion diseases. Int J Mol Sci 10: 976-999.
24. Kovacs GG, Seguin J, Quadrio I, Hoftberger R, Kapas I, et al. (2010) Genetic Creutzfeldt-Jakob disease associated with the E200K mutation: characterization of a complex proteinopathy. Acta Neuropathol 121: 39-57.
25. Baringer JR, Prusiner SB, (1978) Experimental scrapie in mice: ultrastructural observations. Ann Neurol 4: 205-211.
26. Canello T, Frid K, Gabizon R, Lisa S, Friedler A, et al. (2010) Oxidation of Helix-3 methionines precedes the formation of PK resistant PrP. PLoS Pathog 6: e1000977.
27. Pan KM, Stahl N, Prusiner SB, (1992) Purification and properties of the cellular prion protein from Syrian hamster brain. Protein Sci 1: 1343-1352.
28. Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, et al. (1995) Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell 83: 79-90.
29. Shmerling D, Hegyi I, Fischer M, Blattler T, Brandner S, et al. (1998) Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell 93: 203-214.
30. Lasmezas CI, Deslys JP, Robain O, Jaegly A, Beringue V, et al. (1997) Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein. Science 275: 402-405.
31. Legname G, Nguyen HO, Baskakov IV, Cohen FE, Dearmond SJ, et al. (2005) Strain-specified characteristics of mouse synthetic prions. Proc Natl Acad Sci U S A 102: 2168-2173.
32. Harvey BK, Richie CT, Hoffer BJ, Airavaara M, (2010) Transgenic animal models of neurodegeneration based on human genetic studies. J Neural Transm 118: 27-45.
33. Ashe KH, Zahs KR, (2010) Probing the biology of Alzheimer's disease in mice. Neuron 66: 631-645.
34. Harris DA, Chiesa R, Drisaldi B, Quaglio E, Migheli A, et al. (2000) A transgenic model of a familial prion disease. Arch Virol Suppl pp. 103-112.
35. Zanusso G, Farinazzo A, Prelli F, Fiorini M, Gelati M, et al. (2004) Identification of distinct N-terminal truncated forms of prion protein in different Creutzfeldt-Jakob disease subtypes. J Biol Chem 279: 38936-38942.
36. Capellari S, Parchi P, Russo CM, Sanford J, Sy MS, et al. (2000) Effect of the E200K mutation on prion protein metabolism. Comparative study of a cell model and human brain. Am J Pathol 157: 613-622.
37. Simon ES, Kahana E, Chapman J, Treves TA, Gabizon R, et al. (2000) Creutzfeldt-Jakob disease profile in patients homozygous for the PRNP E200K mutation. Ann Neurol 47: 257-260.
38. Forloni G, Salmona M, Marcon G, Tagliavini F, (2009) Tetracyclines and prion infectivity. Infect Disord Drug Targets 9: 23-30.
39. Haviv Y, Avrahami D, Ovadia H, Ben-Hur T, Gabizon R, et al. (2008) Induced neuroprotection independently from PrPSc accumulation in a mouse model for prion disease treated with simvastatin. Arch Neurol 65: 762-775.
40. Ryou C, Legname G, Peretz D, Craig JC, Baldwin MA, et al. (2003) Differential inhibition of prion propagation by enantiomers of quinacrine. Lab Invest 83: 837-843.
41. Peretz D, Williamson RA, Kaneko K, Vergara J, Leclerc E, et al. (2001) Antibodies inhibit prion propagation and clear cell cultures of prion infectivity. Nature 412: 739-743.
42. White MD, Mallucci GR, (2009) RNAi for the treatment of prion disease: a window for intervention in neurodegeneration? CNS Neurol Disord Drug Targets 8: 342-352.
43. Bueler H, Fischer M, Lang Y, Bluethmann H, Lipp HP, et al. (1992) Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature 356: 577-582.
44. Scott M, Groth D, Foster D, Torchia M, Yang SL, et al. (1993) Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes. Cell 73: 979-988.
45. Stahel PF, Shohami E, Younis FM, Kariya K, Otto VI, et al. (2000) Experimental closed head injury: analysis of neurological outcome, blood-brain barrier dysfunction, intracranial neutrophil infiltration, and neuronal cell death in mice deficient in genes for pro-inflammatory cytokines. J Cereb Blood Flow Metab 20: 369-380.
46. Piro JR, Harris BT, Nishina K, Soto C, Morales R, et al. (2009) Prion protein glycosylation is not required for strain-specific neurotropism. J Virol 83: 5321-5328.
47. Tzaban S, Friedlander G, Schonberger O, Horonchik L, Yedidia Y, et al. (2002) Protease-sensitive scrapie prion protein in aggregates of heterogeneous sizes. Biochemistry 41: 12868-12875.