Regional and phenotype heterogeneity of cellular prion proteins in the human brain

European Journal of Neuroscience

Volumn 25, Issue 9, 2007, Pages 2649-2655

  Kuczius, Thorsten ^a   Koch, Richard ^a   Keyvani, Kathy ^a   Karch, Helge ^a   Grassi, Jacques ^b   Groschup, Martin H ^c  

^a UNIVERSITY HOSPITAL MÜNSTER   (Germany)

^b Institut National de la Recherche Agronomique   (France)

^c FEDERAL RESEARCH INSTITUTE FOR ANIMAL HEALTH   (Germany)

Author keywords

Banding pattern; Brain; Distribution; Glycotyping; PrPC

Indexed keywords

CELL PROTEIN; PRION PROTEIN; PROTEIN ANTIBODY;

AMINO TERMINAL SEQUENCE; ANTIGEN BINDING; ARTICLE; BRAIN; BRAIN REGION; CARBOXY TERMINAL SEQUENCE; CEREBELLUM; CONTROLLED STUDY; DENSITOMETRY; FRONTAL CORTEX; GLYCOSYLATION; HIPPOCAMPUS; HUMAN; HUMAN TISSUE; IMMUNOBLOTTING; MOLECULAR SIZE; NEOCORTEX; PHENOTYPE; PRIORITY JOURNAL; PROTEIN DETERMINATION; PROTEIN EXPRESSION; PROTEIN PROCESSING; QUANTITATIVE ANALYSIS; REPEAT PROCEDURE; REPRODUCIBILITY; SIGNAL TRANSDUCTION; STAINING; THALAMUS;

ADULT; AGED; ANIMALS; ANTIBODIES; BRAIN; BRAIN CHEMISTRY; FEMALE; GLYCOPROTEINS; GLYCOSYLATION; HUMANS; IMMUNOHISTOCHEMISTRY; MALE; MICE; MIDDLE AGED; MOLECULAR WEIGHT; PRION DISEASES; PROTEIN ISOFORMS; PROTEIN STRUCTURE, TERTIARY; PRPC PROTEINS;

EID: 34250022898     PISSN: 0953816X     EISSN: 14609568     Source Type: Journal    
DOI: 10.1111/j.1460-9568.2007.05518.x     Document Type: Article

References (42)

1. Asante, E.A., Linehan, J.M., Desbruslais, M., Joiner, S., Gowland, I., Wood, A.L., Welch, J., Hill, A.F., Lloyd, S.E., Wadsworth, J.D. Collinge, J. (2002) BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J., 21, 6358 6366.
2. Bendheim, P.E., Brown, H.R., Rudelli, R.D., Scala, L.J., Goller, N.L., Wen, G.Y., Kascsak, R.J., Cashman, N.R. Bolton, D.C. (1992) Nearly ubiquitous tissue distribution of the scrapie agent precursor protein. Neurology, 42, 149 156.
3. Beringue, V., Mallinson, G., Kaisar, M., Tayebi, M., Sattar, Z., Jackson, G., Anstee, D., Collinge, J. Hawke, S. (2003) Regional heterogeneity of cellular prion protein isoforms in the mouse brain. Brain, 126, 2065 2073.
4. Bolton, D.C., Meyer, R.K. Prusiner, S.B. (1985) Scrapie PrP 27-30 is a sialoglycoprotein. J. Virol., 53, 596 606.
5. Bruce, M.E., McConnell, I., Fraser, H. Dickinson, A.G. (1991) The disease characteristics of different strains of scrapie in Sinc congenic mouse lines: implications for the nature of the agent and host control of pathogenesis. J. Gen. Virol., 72, 595 603.
6. Bruce, M.E., Will, R.G., Ironside, J.W., McConnell, I., Drummond, D., Suttle, A., McCardle, L., Chree, A., Hope, J., Birkett, C., Cousens, S., Fraser, H. Bostock, C.J. (1997) Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature, 389, 498 501.
7. Budka, H. (2000) Histopathology and immunohistochemistry of human transmissible spongiform encephalopathies (TSEs). Arch. Virol. Suppl., 16, 135 142.
8. Budka, H. (2003) Neuropathology of prion diseases. Br. Med. Bull., 66, 121 130.
9. Chen, S.G., Teplow, D.B., Parchi, P., Teller, J.K., Gambetti, P. Autilio-Gambetti, L. (1995) Truncated forms of the human prion protein in normal brain and in prion diseases. J. Biol. Chem., 270, 19173 19780.
10. Collinge, J., Sidle, K.C.L., Meads, J., Ironside, J. Hill, A.F. (1996) Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature, 383, 685 690.
11. Collinge, J., Whittington, M.A., Sidle, K.C., Smith, C.J., Palmer, M.S., Clarke, A.R. Jefferys, J.G. (1994) Prion protein is necessary for normal synaptic function. Nature, 370, 295 297.
12. DeArmond, S.J., Qiu, Y., Sanchez, H., Spilman, P.R., Ninchak-Casey, A., Alonso, D. Daggett, V. (1999) PrPc glycoform heterogeneity as a function of brain region: implications for selective targeting of neurons by prion strains. J. Neuropathol. Exp. Neurol., 58, 1000 1009.
13. Demart, S., Fournier, J.G., Creminon, C., Frobert, Y., Lamoury, F., Marce, D., Lasmezas, C., Dormont, D., Grassi, J. Deslys, J.P. (1999) New insight into abnormal prion protein using monoclonal antibodies. Biochem. Biophys. Res. Commun., 265, 652 657.
14. Endo, T., Groth, D., Prusiner, S.B. Kobata, A. (1989) Diversity of oligosaccharide structures linked to asparagines of the scrapie prion protein. Biochemistry, 28, 8380 8388.
15. Gambetti, P., Kong, Q., Zou, W., Parchi, P. Chen, S.G. (2003) Sporadic and familial CJD: classification and characterisation. Br. Med. Bull., 66, 213 239.
16. Herms, J., Tings, T., Gall, S., Madlung, A., Giese, A., Siebert, H., Schurmann, P., Windl, O., Brose, N. Kretzschmar, H. (1999) Evidence of presynaptic location and function of the prion protein. J. Neurosci., 19, 8866 8875.
17. Hill, A.F. Collinge, J. (2003) Subclinical prion infection in humans and animals. Br. Med. Bull., 66, 161 170.
18. Hope, J., Morton, L.J., Farquhar, C.F., Multhaup, G., Beyreuther, K. Kimberlin, R.H. (1986) The major polypeptide of scrapie-associated fibrils (SAF) has the same size, charge distribution and N-terminal protein sequence as predicted for the normal brain protein (PrP). EMBO J., 5, 2591 2597.
19. Jimenez-Huete, A., Lievens, P.M., Vidal, R., Piccardo, P., Ghetti, B., Tagliavini, F., Frangione, B. Prelli, F. (1998) Endogenous proteolytic cleavage of normal and disease-associated isoforms of the human prion protein in neural and non-neural tissues. Am. J. Pathol., 153, 1561 1572.
20. Krasemann, S., Groschup, M.H., Harmeyer, S., Hunsmann, G. Bodemer, W. (1996) Generation of monoclonal antibodies against human prion proteins in PrP0/0 mice. Mol. Med., 2, 725 734.
21. Kretzschmar, H.A., Prusiner, S.B., Stowring, L.E. DeArmond, S.J. (1986) Scrapie prion proteins are synthesized in neurons. Am. J. Pathol., 122, 1 5.
22. Kuczius, T., Buschmann, A., Zhang, W., Karch, H., Becker, K., Peters, G. Groschup, M.H. (2004) Cellular prion protein acquires resistance to proteolytic degradation following copper ion binding. Biol. Chem., 385, 739 747.
23. Laffont-Proust, I., Faucheux, B.A., Hassig, R., Sazdovitch, V., Simon, S., Grassi, J., Hauw, J.J., Moya, K.L. Haik, S. (2005) The N-terminal cleavage of cellular prion protein in the human brain. FEBS Lett., 579, 6333 6337.
24. Lasmezas, C.I. (2003) Putative functions of PrPC. Br. Med. Bull., 66, 61 70.
25. Liu, T., Zwingman, T., Li, R., Pan, T., Wong, B.S., Petersen, R.B., Gambetti, P., Herrup, K. Sy, M.S. (2001) Differential expression of cellular prion protein in mouse brain as detected with multiple anti-PrP monoclonal antibodies. Brain Res., 896, 118 129.
26. Maglio, L.E., Perez, M.F., Martins, V.R., Brentani, R.R. Ramirez, O.A. (2004) Hippocampal synaptic plasticity in mice devoid of cellular prion protein. Brain Res. Mol. Brain Res., 131, 58 64.
27. 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, 117 122.
28. Markwell, M.A., Haas, S.M., Bieber, L.L. Tolbert, N.E. (1978) A modification of the Lowry procedure to simplify protein determination in membrane and lipoprotein samples. Anal. Biochem., 87, 206 210.
29. McLennan, N.F., Rennison, K.A., Bell, J.E. Ironside, J.W. (2001) In situ hybridization analysis of PrP mRNA in human CNS tissues. Neuropathol. Appl. Neurobiol., 27, 373 383.
30. Moleres, F.J. Velayos, J.L. (2005) Expression of PrPC in the rat brain and characterization of a subset of cortical neurons. Brain Res., 1056, 10 21.
31. Mouillet-Richard, S., Ermonval, M., Chebassier, C., Laplanche, J.L., Lehmann, S., Launay, J.M. Kellermann, O. (2000) Signal transduction through prion protein. Science, 289, 1925 1928.
32. Moya, K.L., Sales, N., Hassig, R., Creminon, C., Grassi, J. Di Giamberardino, L. (2000) Immunolocalization of the cellular prion protein in normal brain. Microsc. Res. Techn., 50, 58 65.
33. Polymenidou, M., Stoeck, K., Glatzel, M., Vey, M., Bellon, A. Aguzzi, A. (2005) Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease. Lancet Neurol., 4, 805 814.
34. Prusiner, S.B. (1991) Molecular biology of prion diseases. Science, 252, 1515 1522.
35. Robakis, N.K., Devine-Gage, E.A., Jenkins, E.C., Kascsak, R.J., Brown, W.T., Krawczun, M.S. Silverman, W.P. (1986) Localization of a human gene homologous to the PrP gene on the p arm of chromosome 20 and detection of PrP-related antigens in normal human brain. Biochem. Biophys. Res. Commun., 140, 758 765.
36. Sales, N., Rodolfo, K., Hassig, R., Faucheux, B., Di Giamberardino, L. Moya, K.L. (1998) Cellular prion protein localization in rodent and primate brain. Eur. J. Neurosci., 10, 2464 2471.
37. Stahl, N., Baldwin, M.A., Hecker, R., Pan, K.M., Burlingame, A.L. Prusiner, S.B. (1992) Glycosylinositol phospholipid anchors of the scrapie and cellular prion proteins contain sialic acid. Biochemistry, 31, 5043 5053.
38. Stahl, N., Borchelt, D.R. Prusiner, S.B. (1990) Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C. Biochemistry, 29, 5405 5412.
39. Taraboulos, A., Jendroska, K., Serban, D., Yang, S.L., DeArmond, S.J. Prusiner, S.B. (1992) Regional mapping of prion proteins in brain. Proc. Natl Acad. Sci. USA, 89, 7620 7624.
40. Vorberg, I. Priola, S.A. (2002) Molecular basis of scrapie strain glycoform variation. J. Biol. Chem., 277, 36775 36781.
41. Wion, D., Le Bert, M. Brachet, P. (1988) Messenger RNAs of beta-amyloid precursor protein and prion protein are regulated by nerve growth factor in PC12 cells. Int. J. Dev. Neurosci., 6, 387 393.
42. Yull, H.M., Ritchie, D.L., Langeveld, J.P., van Zijderveld, F.G., Bruce, M.E., Ironside, J.W. Head, M.W. (2006) Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease. Am. J. Pathol., 168, 151 157.