How many phenotypes from one genotype? The case of prion diseases

Journal of Theoretical Biology

Volumn 182, Issue 3, 1996, Pages 209-218

  Kacser, Henrik ^b   Small, J Rankin ^a,b  

^a University of Edinburgh ^*  (United Kingdom)

^b UNIVERSITY OF EDINBURGH   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

DISEASE TRANSMISSION; GENOTYPE; METABOLIC REGULATION; PHENOTYPE; PRION DISEASE; PRIORITY JOURNAL; REVIEW; SHEEP DISEASE; STEADY STATE;

OVIS ARIES;

EID: 0030574088     PISSN: 00225193     EISSN: None     Source Type: Journal    
DOI: 10.1006/jtbi.1996.0157     Document Type: Article

References (34)

1. BASLER, K., OESCH, B., SCOTT, M., WESTAWAY, D., WÄLCHI, M. et al. (1986). Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell 46, 417-428.
2. BENDHEIM, P. E. & BOLTON, D. C. (1991). The transmissible agent causing scrapic must contain more than protein. Reviews in Medical Virology 1, 131-144.
3. BRUCE, M. E. (1985). Agent replication dynamics in a long incubation period model of mouse scrapie. J. Gen. Virol. 66, 2517-2522.
4. BRUCE, M. E. & DICKINSON, A. G. (1987). Biological evidence that scrapie agent has an independent genome. J. Gen. Virol. 68, 79-89.
5. BRUCE, M. E. & FRASER, H. (1991). Scrapie strain variation and its implications. Curr. Topics in Microbio. and Enzymol. 172, 125-138.
6. 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.
7. BÜLER, H., AGUZZI, A., SAILER, A., GREINER, R. A., AUTENREID, P., AGNET, M. & WEISSMANN, C. (1993). Mice devoid of PrP are resistant to scrapie. Cell 73, 1339-1347.
8. CARLSON, G. A., HSIAO, K., OESCH, B., WESTAWAY, D. & PRUSINER, S. B. (1991). Genetics of prion infections. TIG 7, 61-65.
9. CAUGHEY, B. & GREGORY, J. R. (1993). Sulfated polyanion inhibition of Scrapie-associated PrP accumulation in cultured cells. J. Virol. 67, 643-650.
10. CAUGHEY, B., ERNST, D. & RACE, R. E. (1993). Congo Red inhibition of Scrapie Agent replication. J. Virol. 67, 6270-6272.
11. COHEN, F. E., PAN, K.-M., HUANG, Z., BALDWIN, M., FLETTERICK, R. J. & PRUSINER, S. B. (1994). Structural clues to prion replication. Science 264, 530-531.
12. DECROLY, O. & GOLDBETER, A. (1982). Birhythmicity, chaos, and other patterns of temporal self-organization in a multiply regulated biochemical system. P.N.A.S. 79, 6917-6921.
13. DICKINSON, A. G., BRUCE, M. E., OUTROUN, G. W. & KIMBERLIN, R. H. (1984). Scrapie strain differences: the implications of stability and mutation. In: Proceedings of Workshop on Slow Transmissible Diseases (Tateishi, J., ed.) pp. 105-118. Tokyo: Japanese Ministry of Health & Welfare.
14. DIRINGER, D. & EHLERS, B. (1991). Chemoprophylaxis of scrapie in mice. J. Gen. Virol. 72, 457-460.
15. FELL, D. A. (1992). Metabolic Control Analysis: a survey of its theoretical and experimental development. Biochem. J. 286, 313-330.
16. FRASER, H., BRUCE, M. E., CHREE, A., MCCONNELL, I. & WELLS, G. A. H. (1992). Transmission of bovine spongiform encephalopathy and scrapie to mice. J. Gen. Virol. 73, 1891-1897.
17. GOLDBETER, A. & DUPONT, G. (1990). Allosteric Regulation, Cooperativity and Biochemical Oscillations. Biophys. Chem. 37, 341-353.
18. GOLDBETER, A. & MORAN, F. (1987). Complex patterns of excitability and oscillation in a biochemical system. In: The Organization of Cell Metabolism (Welch, G. R. & Clegg, J. S., eds) pp. 291-305. New York: Plenum.
19. GOLDBETER, A. & WOLPERT, L. (1990). Covalent modification of proteins as a threshold mechanism in development. J. theor. Biol. 142, 243-250.
20. KACSER, H. (1987). On parts and wholes in metabolism. In: The Organisation of Cell Metabolism (Welch, G. R. & Clegg, J. S., eds.) pp. 327-337. New York: Plenum.
21. KACSER, H. & BURNS, J. A. (1979). Molecular democracy: who shares the control. Biochem. Soc. Trans. 7, 1149-1160.
22. KACSER, H. & BURNS, J. A. (1981). The molecular basis of dominance. Genetics 97, 69-666.
23. KEIGHTLEY, P. D. (1989). Models of quantitative variation of flux in metabolic pathways. Genetics 121, 869-876.
24. KEIGHTLEY, P. D. & KACSER, H. (1987). Dominance, pleiotropy and metabolic structure. Genetics 117, 313-329.
25. KELLER, A. D. (1995). Model genetic circuits encoding autoregulatory transcription factors. J. theor. Biol. 172, 169-185.
26. PRUSINER, S. B. (1991). Molecular biology of prion diseases. Science 252, 1515-1522.
27. REICH, J. G. & SEL'KOV, E. E. (1981). Energy Metabolism of the Cell: A Theoretical Treatise. New York: Academic Press.
28. RIESNER, D. (1991). The search for a nucleic acid component to scrapie infectivity. Virology 2, 215-216.
29. SAURO, H. M. (1993). SCAMP: A general purpose simulator and metabolic control analysis program. Comput. Appl. Biosci. 9, 441-450.
30. SCOTT, J. R., REEKIE, L. J. D. & HOPE, J. (1991). Evidence for the intrinsic control of scrapie pathogenesis in the murine visual system. Neuroscience Letters 133, 141-144.
31. STAAHL, N., BALDWIN, M. A., TEPLOW, D. B., HOOD, L., GIBSON, B. W. et al. (1993). Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing. Biochemistry 32, 1991-2002.
32. TUITE, M. E. (1994). Psi no more for yeast prions. Nature 370, 327-328.
33. WEISSMANN, C. (1991). A "unified theory" of prion propagation. Nature 352, 679-683.
34. WOLFRAM, S. (1991). Mathematica: A System for Doing Mathematics by Computer, 2nd Edn. California: Addison-Wesley.