Requirements for mutant and wild-type prion protein misfolding in vitro

Biochemistry

Volumn 54, Issue 5, 2015, Pages 1180-1187

  Noble, Geoffrey P ^a   Walsh, Daniel J ^a   Miller, Michael B ^a,c   Jackson, Walker S ^b   Supattapone, Surachai ^a  

^a DARTMOUTH MEDICAL SCHOOL   (United States)

^b GERMAN CENTER FOR NEURODEGENERATIVE DISEASES DZNE   (Germany)

^c HARVARD MEDICAL SCHOOL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

MOLECULES; NEURODEGENERATIVE DISEASES; PHOSPHOLIPIDS; PROTEINS;

CELLULAR FACTORS; DOSE-DEPENDENT MANNER; INFECTIOUS PRIONS; MISFOLDING; PRION DISEASE; PRION PROPAGATION; PRION PROTEIN; WILD TYPES;

DISEASE CONTROL;

MUTANT PROTEIN; PHOSPHOLIPID; PRION PROTEIN; PRION;

ANIMAL TISSUE; ARTICLE; CONFORMATION; DOSE RESPONSE; EPITOPE MAPPING; IN VITRO STUDY; MUTATION; NONHUMAN; PH; PRION DISEASE; PRIORITY JOURNAL; PROTEIN FOLDING; PROTEIN MISFOLDING; PROTEIN PROTEIN INTERACTION; PROTEIN STRUCTURE; RAT; SUBSTITUTION REACTION; TEMPERATURE; ULTRASOUND; WILD TYPE; AMINO ACID SUBSTITUTION; ANIMAL; CHEMISTRY; GENETICS; HEAT; METABOLISM; MISSENSE MUTATION; MOUSE; PRION;

AMINO ACID SUBSTITUTION; ANIMALS; HOT TEMPERATURE; HYDROGEN-ION CONCENTRATION; MICE; MUTATION, MISSENSE; PRION DISEASES; PRIONS; PROTEIN FOLDING;

EID: 84922634099     PISSN: 00062960     EISSN: 15204995     Source Type: Journal    
DOI: 10.1021/bi501495j     Document Type: Article

References (42)

1. Prusiner, S. B. (1998) Prions Proc. Natl. Acad. Sci. U. S. A. 95, 13363-13383
2. Kocisko, D. A., Come, J. H., Priola, S. A., Chesebro, B., Raymond, G. J., Lansbury, P. T., and Caughey, B. (1994) Cell-free formation of protease-resistant prion protein Nature 370, 471-474
3. Castilla, J., Saa, P., Hetz, C., and Soto, C. (2005) In vitro generation of infectious scrapie prions Cell 121, 195-206
4. Deleault, N. R., Harris, B. T., Rees, J. R., and Supattapone, S. (2007) Formation of native prions from minimal components in vitro Proc. Natl. Acad. Sci. U. S. A. 104, 9741-9746
5. Deleault, N. R., Piro, J. R., Walsh, D. J., Wang, F., Ma, J., Geoghegan, J. C., and Supattapone, S. (2012) Isolation of phosphatidylethanolamine as a solitary cofactor for prion formation in the absence of nucleic acids Proc. Natl. Acad. Sci. U. S. A. 109, 8546-8551
6. Deleault, N. R., Walsh, D. J., Piro, J. R., Wang, F., Wang, X., Ma, J., Rees, J. R., and Supattapone, S. (2012) Cofactor molecules maintain infectious conformation and restrict strain properties in purified prions Proc. Natl. Acad. Sci. U. S. A. 109, E1938-E1946
7. Gambetti, P., Parchi, P., Petersen, R. B., Chen, S. G., and Lugaresi, E. (1995) Fatal familial insomnia and familial Creutzfeldt-Jakob disease: clinical, pathological and molecular features Brain Pathol. 5, 43-51
8. Hsiao, K., Meiner, Z., Kahana, E., Cass, C., Kahana, I., Avrahami, D., Scarlato, G., Abramsky, O., Prusiner, S. B., and Gabizon, R. (1991) Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease N. Engl. J. Med. 324, 1091-1097
9. Jackson, W. S., Borkowski, A. W., Faas, H., Steele, A. D., King, O. D., Watson, N., Jasanoff, A., and Lindquist, S. (2009) Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice Neuron 63, 438-450
10. Jackson, W. S., Borkowski, A. W., Watson, N. E., King, O. D., Faas, H., Jasanoff, A., and Lindquist, S. (2013) Profoundly different prion diseases in knock-in mice carrying single PrP codon substitutions associated with human diseases Proc. Natl. Acad. Sci. U. S. A. 110, 14759-14764
11. Breydo, L., Makarava, N., and Baskakov, I. V. (2008) Methods for conversion of prion protein into amyloid fibrils Methods Mol. Biol. 459, 105-115
12. Walsh, D. J., Noble, G. P., Piro, J. R., and Supattapone, S. (2012) Non-reducing alkaline solubilization and rapid on-column refolding of recombinant prion protein Prep. Biochem. Biotechnol. 42, 77-86
13. Owen, F., Poulter, M., Collinge, J., and Crow, T. J. (1990) A codon 129 polymorphism in the PRIP gene Nucleic Acids Res. 18, 3103
14. Williamson, R. A., Peretz, D., Pinilla, C., Ball, H., Bastidas, R. B., Rozenshteyn, R., Houghten, R. A., Prusiner, S. B., and Burton, D. R. (1998) Mapping the prion protein using recombinant antibodies J. Virol. 72, 9413-9418
15. Spinner, D. S., Kascsak, R. B., Lafauci, G., Meeker, H. C., Ye, X., Flory, M. J., Kim, J. I., Schuller-Levis, G. B., Levis, W. R., Wisniewski, T., Carp, R. I., and Kascsak, R. J. (2007) CpG oligodeoxynucleotide-enhanced humoral immune response and production of antibodies to prion protein PrPSc in mice immunized with 139A scrapie-associated fibrils J. Leukoc. Biol. 81, 1374-1385
16. Wang, F., Wang, X., Yuan, C. G., and Ma, J. (2010) Generating a Prion with Bacterially Expressed Recombinant Prion Protein Science 327, 1132-1135
17. Brown, P., Gibbs, C. J., Jr., Rodgers-Johnson, P., Asher, D. M., Sulima, M. P., Bacote, A., Goldfarb, L. G., and Gajdusek, D. C. (1994) Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease Ann. Neurol. 35, 513-529
18. Prusiner, S. B. (1994) Inherited prion diseases Proc. Natl. Acad. Sci. U. S. A. 91, 4611-4614
19. Will, R. G., Alperovitch, A., Poser, S., Pocchiari, M., Hofman, A., Mitrova, E., de Silva, R., DAlessandro, M., Delasnerie-Laupretre, N., Zerr, I., and van Duijn, C. (1998) Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993-1995. EU Collaborative Study Group for CJD Ann. Neurol. 43, 763-767
20. Lee, S., Antony, L., Hartmann, R., Knaus, K. J., Surewicz, K., Surewicz, W. K., and Yee, V. C. (2010) Conformational diversity in prion protein variants influences intermolecular beta-sheet formation EMBO J. 29, 251-262
21. Liemann, S. and Glockshuber, R. (1999) Influence of amino acid substitutions related to inherited human prion diseases on the thermodynamic stability of the cellular prion protein Biochemistry 38, 3258-3267
22. Apetri, A. C., Vanik, D. L., and Surewicz, W. K. (2005) Polymorphism at residue 129 modulates the conformational conversion of the D178N variant of human prion protein 90-231 Biochemistry 44, 15880-15888
23. Gao, C., Ren, K., Li, L. Z., Jiang, H. Y., Chen, C., Zhang, J., Han, J., and Dong, X. P. (2012) Mutant D178N prion protein converts spontaneously in RT-QuIC assay Zhonghua Shi Yan He Lin Chuang Bing Du Xue Za Zhi 26, 370-373 [in Chinese]
24. el-Bastawissy, E., Knaggs, M. H., and Gilbert, I. H. (2001) Molecular dynamics simulations of wild-type and point mutation human prion protein at normal and elevated temperature J. Mol. Graph. Model. 20, 145-154
25. Gsponer, J., Ferrara, P., and Caflisch, A. (2001) Flexibility of the murine prion protein and its Asp178Asn mutant investigated by molecular dynamics simulations J. Mol. Graph. Model. 20, 169-182
26. Meli, M., Gasset, M., and Colombo, G. (2011) Dynamic diagnosis of familial prion diseases supports the beta2-alpha2 loop as a universal interference target PLoS One 6, e19093
27. Shamsir, M. S. and Dalby, A. R. (2005) One gene, two diseases and three conformations: molecular dynamics simulations of mutants of human prion protein at room temperature and elevated temperatures Proteins 59, 275-290
28. Castilla, J., Saa, P., Morales, R., Abid, K., Maundrell, K., and Soto, C. (2006) Protein misfolding cyclic amplification for diagnosis and prion propagation studies Methods Enzymol. 412, 3-21
29. Kitamoto, T., Iizuka, R., and Tateishi, J. (1993) An amber mutation of prion protein in Gerstmann-Straussler syndrome with mutant PrP plaques Biochem. Biophys. Res. Commun. 192, 525-531
30. Muramoto, T., Tanaka, T., Kitamoto, N., Sano, C., Hayashi, Y., Kutomi, T., Yutani, C., and Kitamoto, T. (2000) Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu Neurosci. Lett. 288, 179-182
31. Tagliavini, F., Prelli, F., Porro, M., Rossi, G., Giaccone, G., Farlow, M. R., Dlouhy, S. R., Ghetti, B., Bugiani, O., and Frangione, B. (1994) Amyloid fibrils in Gerstmann-Straussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele Cell 79, 695-703
32. Chen, S. G., Parchi, P., Brown, P., Capellari, S., Zou, W., Cochran, E. J., Vnencak-Jones, C. L., Julien, J., Vital, C., Mikol, J., Lugaresi, E., Autilio-Gambetti, L., and Gambetti, P. (1997) Allelic origin of the abnormal prion protein isoform in familial prion diseases Nat. Med. 3, 1009-1015
33. Mastrianni, J. A., Nixon, R., Layzer, R., Telling, G. C., Han, D., DeArmond, S. J., and Prusiner, S. B. (1999) Prion protein conformation in a patient with sporadic fatal insomnia N. Engl J. Med. 340, 1630-1638
34. Gabizon, R., Telling, G., Meiner, Z., Halimi, M., Kahana, I., and Prusiner, S. B. (1996) Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease Nat. Med. 2, 59-64
35. Silvestrini, M. C., Cardone, F., Maras, B., Pucci, P., Barra, D., Brunori, M., and Pocchiari, M. (1997) Identification of the prion protein allotypes which accumulate in the brain of sporadic and familial Creutzfeldt-Jakob disease patients Nat. Med. 3, 521-525
36. Wadsworth, J. D., Joiner, S., Linehan, J. M., Cooper, S., Powell, C., Mallinson, G., Buckell, J., Gowland, I., Asante, E. A., Budka, H., Brandner, S., and Collinge, J. (2006) Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein Brain: J. Neurol. 129, 1557-1569
37. Monari, L., Chen, S. G., Brown, P., Parchi, P., Petersen, R. B., Mikol, J., Gray, F., Cortelli, P., Montagna, P., and Ghetti, B. 1994, Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism Proc. Natl. Acad. Sci. U. S. A. 91, 2839-2842
38. Telling, G. C., Parchi, P., DeArmond, S. J., Cortelli, P., Montagna, P., Gabizon, R., Mastrianni, J., Lugaresi, E., Gambetti, P., and Prusiner, S. B. (1996) Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity Science 274, 2079-2082
39. Geoghegan, J. C., Miller, M. B., Kwak, A. H., Harris, B. T., and Supattapone, S. (2009) Trans-dominant inhibition of prion propagation in vitro is not mediated by an accessory cofactor PLoS Pathog. 5, e1000535
40. Lee, C. I., Yang, Q., Perrier, V., and Baskakov, I. V. (2007) The dominant-negative effect of the Q218K variant of the prion protein does not require protein X Protein Sci. 16, 2166-2173
41. Krasnianski, A., Bartl, M., Sanchez Juan, P. J., Heinemann, U., Meissner, B., Varges, D., Schulze-Sturm, U., Kretzschmar, H. A., Schulz-Schaeffer, W. J., and Zerr, I. (2008) Fatal familial insomnia: Clinical features and early identification Ann. Neurol. 63, 658-661
42. Manetto, V., Medori, R., Cortelli, P., Montagna, P., Tinuper, P., Baruzzi, A., Rancurel, G., Hauw, J. J., Vanderhaeghen, J. J., and Mailleux, P. 1992, Fatal familial insomnia: clinical and pathologic study of five new cases Neurology 42, 312-319