Identification of a novel gene encoding a PrP-like protein expressed as chimeric transcripts fused to PrP exon 1/2 in ataxic mouse line with a disrupted PrP gene

Cellular and Molecular Neurobiology

Volumn 20, Issue 5, 2000, Pages 553-567

  Li, Aimin ^a   Sakaguchi, Suehiro ^a   Atarashi, Ryuichiro ^a   Roy, Bhabesh C ^a   Nakaoke, Ryota ^a   Arima, Kazuhiko ^a   Okimura, Nobuhiko ^a   Kopacek, Juraj ^a,b   Shigematsu, Kazuto ^a  

^a NAGASAKI UNIVERSITY SCHOOL OF MEDICINE   (Japan)

^b INSTITUTE OF VIROLOGY   (Slovakia)

Author keywords

Ataxia; Knockout mice; Prion protein; Prion protein like protein; Purkinje cell

Indexed keywords

MESSENGER RNA; PRION PROTEIN;

AMINO ACID SEQUENCE; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; ATAXIA; CHIMERA; CONTROLLED STUDY; EXON; GENE AMPLIFICATION; GENE DISRUPTION; GENE EXPRESSION; IN SITU HYBRIDIZATION; INTRON; KNOCKOUT MOUSE; MOUSE; NERVE CELL DEGENERATION; NONHUMAN; NORTHERN BLOTTING; NUCLEOTIDE SEQUENCE; PRIORITY JOURNAL; PROMOTER REGION; PURKINJE CELL; PYRAMIDAL NERVE CELL; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION;

AMINO ACID SEQUENCE; ANIMALS; ATAXIA; BASE SEQUENCE; BLOTTING, NORTHERN; BRAIN CHEMISTRY; EXONS; GENE EXPRESSION; INTRONS; MICE; MICE, KNOCKOUT; MICE, NEUROLOGIC MUTANTS; MOLECULAR SEQUENCE DATA; PHENOTYPE; PRIONS; PROMOTER REGIONS (GENETICS); PRPC PROTEINS; PURKINJE CELLS; RECOMBINANT FUSION PROTEINS; RNA, MESSENGER; TRANSCRIPTION, GENETIC;

ANIMALIA; ATAXIA;

EID: 0033942010     PISSN: 02724340     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1007059827541     Document Type: Article

References (20)

1. Antoniou, M., Geraghty, F., Hurst, J., and Grosveld, F. (1998). Efficient 3′-end formation of human β-globin mRNA in vivo requires sequences within the last intron but occurs independently of the splicing reaction. Nucleic Acids Res. 26:721-729.
2. Büeler, H., Aguzzi, A., Sailer, A., Greiner, R. A., Autenried, P., Aguet, M., and Weissmann, C. (1993). Mice devoid of PrP are resistant to scrapie. Cell 73:1339-1347.
3. Büeler, H., Fischer, M., Lang, Y., Bluethmann, H., Lipp, H. P., DeArmond, S. J., Prusiner, S. B., Aguet, M., and Weissmann, C. (1992). Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature 356:577-582.
4. Gunderson, S. I., Beyer, K., Martin, G., Keller, W., Boelens, W. C., and Mattaj, L. W. (1994). The human U1AsnRNP protein regulates polyadenylation via a direct interaction with poly(A) polymerase. Cell 76:531-541.
5. Manson, J. C., Clarke, A. R., Hooper, M. L., Aitchison, L., McConnell, I., and Hope, J. (1994a). 129/ Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal. Mol. Neurobiol. 8:121-127.
6. Manson, J. C., Clarke, A. R., McBride, P. A., McConnell, I., and Hope, J. (1994b). PrP gene dosage determines the timing but not the final intensity or distribution of lesions in scrapie pathology. Neurodegeneration 3:331-340.
7. Moore, R. (1997). Gene targeting studies at the mouse prion protein locus [dissertation]. University of Edinburgh, Edinburgh, Scotland.
8. Moore, R. C., Lee, I. Y., Silverman, G. L., Harrison, P. M., Strome, R., Heinrich, C., Karunarantne, A., Pasternak, S. H., Chishti, M. A., Liang, Y., Mastrangelo, P., Wang, K., Smit, A. F. A., Katamine, S., Carlson, G. A., Cohen, F. E., Prusiner, S. B., Melton, A. W., Tremblay, P., Hood, L. E., and Westaway, D. (1999). Ataxia in prion protein (PrP) deficient mice associated with upregulation of the novel PrP-like protein doppel. J. Mol. Biol. 292:797-817.
9. Nishida, N., Tremblay, P., Sugimoto, T., Shigematsu, K., Shirabe, S., Petromilli, C., Erpel, S. P., Nakaoke, R., Atarashi, R., Houtani, T., Torchia, M., Sakaguchi, S., DeArmond, S. J., Prusiner, S. B., and Katamine, S. (1999). A mouse prion protein transgene rescues mice deficient for the prion protein gene from Purkinje cell degeneration and demyelination. Lab. Invest. 79:689-697.
10. Prusiner, S. B. (1991). Molecular biology of prion diseases. Science 252:1515-1522.
11. Prusiner, S. B. (1998). Prions. Proc. Natl. Acad. Sci. USA 95:13363-13383.
12. Prusiner, S. B., Groth, D., Serban, A., Koehler, R., Foster, D., Torchia, M., Burton, D., Yang, S. L., and DeArmond, S. J. (1993). Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. Proc. Natl. Acad. Sci. USA 90:10608-10612.
13. Rieger, R., Edenhofer, F., Lasmezas, C. I., and Weiss, S. (1997). The human 37-kDa laminin receptor precursor interacts with the prion protein in eukaryotic cells. Nat. Med. 3:1383-1388.
14. Riek, R., Hornemann, S., Wider, G., Billeter, M., Glockshuber, R., and Wüthrich, K. (1996). NMR structure of the mouse prion protein domain PrP(121-231). Nature 382:180-182.
15. Sakaguchi, S., Katamine, S., Nishida, N., Moriuchi, R., Shigematsu, K., Sugimoto, T., Nakatani, A., Kataoka, Y., Houtani, T., Shirabe, S., Okada, H., Hasegawa, S., Miyamoto, T., and Noda, T. (1996). Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene. Nature 380:528-531.
16. Sakaguchi, S., Katamine, S., Shigematsu, K., Nakatani, A., Moriuchi, R., Nishida, N., Kurokawa, K., Nakaoke, R., Sato, H., Jishage, K., et al. (1995). Accumulation of proteinase K-resistant prion protein (PrP) is restricted by the expression level of normal PrP in mice inoculated with a mouse-adapted strain of the Creutzfeldt-Jakob disease agent. J. Virol. 69:7586-7592.
17. Shmerling, D., Hegyi, I., Fischer, M., Blattler, T., Brandner, S., Gotz, J., Rulicke, T., Flechsig, E., Cozzio, A., von Mering, C., Hangartner, C., Aguzzi, A., and Weissmann, C. (1998). Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell 93:203-214.
18. Stahl, N., and Prusiner, S. B. (1991). Prions and prion proteins. FASEB J. 5:2799-2807.
19. Steinmetz, E. J. (1997). Pre-mRNA processing and the CTD of RNA polymerase II: The tail that wags the dog? Cell 89:491-494.
20. Thompson, J. D., Higgins, D. G., and Gibson, T. (1994). CLUSTAL W: Improving the sensitivity of progressive multiple sequence alignment through sequence weighting, position-specific gap penalties and weight matrix choice. Nucleic Acids Res. 22:4673-4680.