Dimeric transthyretin variant assembles into spherical neurotoxins

Biochemistry

Volumn 44, Issue 9, 2005, Pages 3280-3288

  Matsubara, Kimiaki ^a   Mizuguchi, Mineyuki ^a   Igarashi, Kouhei ^a   Shinohara, Yoshinori ^b   Takeuchi, Makoto ^a   Matsuura, Atsushi ^a   Saitoh, Takayuki ^a   Mori, Yoshihiro ^a   Shinoda, Hiroyuki ^a   Kawano, Keiichi ^a,c  

^a UNIVERSITY OF TOYAMA   (Japan)

^b KYUSHU UNIVERSITY   (Japan)

^c HOKKAIDO UNIVERSITY   (Japan)

Author keywords

[No Author keywords available]

Indexed keywords

CELLS; DISEASE CONTROL; MUTAGENESIS; PH EFFECTS;

CELL DEATH; CYTOTOXICITY; NEUROTOXINS; POLYNEUROPATHY;

TOXIC MATERIALS;

8 ANILINO 1 NAPHTHALENESULFONIC ACID; ACRYLAMIDE; GLUTARALDEHYDE; NEUROTOXIN; PREALBUMIN; UREA;

ARTICLE; ATOMIC FORCE MICROSCOPY; CELL DEATH; CIRCULAR DICHROISM; CROSS LINKING; CYTOTOXICITY; FLUORESCENCE; GEL PERMEATION CHROMATOGRAPHY; HUMAN; HUMAN CELL; NEUROBLASTOMA CELL; PH; PRIORITY JOURNAL; PROTEIN STABILITY; PROTEIN STRUCTURE; PROTEIN TERTIARY STRUCTURE; SITE DIRECTED MUTAGENESIS;

ACRYLAMIDE; AMINO ACID SUBSTITUTION; ANILINO NAPHTHALENESULFONATES; CELL DEATH; CELL LINE, TUMOR; CHROMATOGRAPHY, GEL; DIMERIZATION; HUMANS; ISOLEUCINE; MICROSCOPY, ATOMIC FORCE; NEPHELOMETRY AND TURBIDIMETRY; NEUROBLASTOMA; NEUROTOXINS; PREALBUMIN; PROTEIN BINDING; PROTEIN CONFORMATION; PROTEIN PROCESSING, POST-TRANSLATIONAL; PROTEIN STRUCTURE, QUATERNARY; PROTEIN STRUCTURE, SECONDARY; PROTEIN STRUCTURE, TERTIARY; SERINE; SPECTROMETRY, FLUORESCENCE; THERMODYNAMICS; TRYPTOPHAN; VARIATION (GENETICS);

EID: 20044385548     PISSN: 00062960     EISSN: None     Source Type: Journal    
DOI: 10.1021/bi048838c     Document Type: Article

References (39)

1. Glenner, G. G. (1980) Amyloid deposits and amyloidosis. The beta-fibrilloses, N. Engl. J. Med. 302, 1283-1292.
2. Naiki, H., Higuchi, K., Hosokawa, M., and Takeda, T. (1989) Fluorometric determination of amyloid fibrils in vitro using the fluorescent dye, thioflavin T1, Anal. Biochem. 177, 244-249.
3. Lansbury, P. T. (1992) In pursuit of the molecular structure of amyloid plaque: new technology provides unexpected and critical information, Biochemistry 31, 6865-6870.
4. Sunde, M., Serpell, L. C., Bartlam, M., Fraser, P. E., Pepys, M. B., and Blake, C. C. (1997) Common core structure of amyloid fibrils by synchrotron X-ray diffraction, J. Mol. Biol. 273, 729-739.
5. Guijarro, J. I., Sunde, M., Jones, J. A., Campbell, I. D., and Dobson, C. M. (1998) Amyloid fibril formation by an SH3 domain, Proc. Natl. Acad. Sci. U.S.A. 95, 4224-4228.
6. Bucciantini, M., Giannoni, E., Chiti, F., Baroni, F., Formigli, L., Zurdo, J., Taddei, N., Ramponi, G., Dobson, C. M., and Stefani, M. (2002) Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases, Nature 416, 507-511.
7. Blake, C. C., Geisow, M. J., Swan, I. D., Rerat, C., and Rerat, B. (1974) Structure of human plasma prealbumin at 2-5 A resolution. A preliminary report on the polypeptide chain conformation, quaternary structure and thyroxine binding, J. Mol. Biol. 88, 1-12.
8. Blake, C. C., Geisow, M. J., Oatley, S. J., Rerat, B., and Rerat, C. (1978) Structure of prealbumin: secondary, tertiary and quaternary interactions determined by Fourier refinement at 1.8 A, J. Mol. Biol. 121, 339-356.
9. Saraiva, M. J., Birken, S., Costa, P. P., and Goodman, D. S. (1984) Amyloid fibril protein in familial amyloidotic polyneuropathy, Portuguese type. Definition of molecular abnormality in transthyretin (prealbumin), J. Clin. Invest. 74, 104-119.
10. Saraiva, M. J., Costa, P. P., and Goodman, D. S. (1988) Transthyretin (prealbumin) in familial amyloidotic polyneuropathy: genetic and functional aspects, Adv. Neurol. 48, 189-200.
11. Eneqvist, T., and Sauer-Eriksson, A. E. (2001) Structural distribution of mutations associated with familial amyloidotic polyneuropathy in human transthyretin, Amyloid 8, 149-168.
12. Connors, L. H., Richardson, A. M., Theberge, R., and Costello, C. E. (2000) Tabulation of transthyretin (TTR) variants as of 1/1/ 2000, Amyloid 7, 54-69.
13. Hund, E., Linke, R. P., Willig, F., and Grau, A. (2001) Transthyretin-associated neuropathic amyloidosis. Pathogenesis and treatment, Neurology 56, 431-435.
14. Colon, W., and Kelly, J. W. (1992) Partial denaturation of transthyretin is sufficient for amyloid fibril formation in vitro, Biochemistry 31, 8654-8660.
15. McCutchen, S. L., Colon, W., and Kelly, J. W. (1993) Transthyretin mutation Leu-55-Pro significantly alters tetramer stability and increases amyloidogenicity, Biochemistry 32, 12119-12127.
16. Lai, Z., Colon, W., and Kelly, J. W. (1996) The acid-mediated denaturation pathway of transthyretin yields a conformational intermediate that can self-assemble into amyloid, Biochemistry 35, 6470-6482.
17. Jiang, X., Smith, C. S., Petrassi, H. M., Hammarstrom, P., White, J. T., Sacchettini, J. C., and Kelly J. W. (2001) An engineered transthyretin monomer that is nonamyloidogenic, unless it is partially denatured, Biochemistry 40, 11442-11452.
18. Walsh, D. M., Klyubin, L. Fadeeva, J. V., Cullen, W. K., Anwyl, R., Wolfe, M. S., Rowan, M. J., and Selkoe, D. J. (2002) Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo, Nature 416, 535-539.
19. Hartley, D. M., Walsh, D. M., Ye, C. P., Diehl, T., Vasquez, S., Vassilev, P. M., Teplow, D. B., and Selkoe, D. J. (1999) Protofibrillar intermediates of amyloid beta-protein induce acute electrophysiological changes and progressive neurotoxicity in cortical neurons, J. Neurosci. 19, 8876-8884.
20. Hoshi, M., Sato, M., Matsumoto, S., Noguchi, A., Yasutake, K., Yoshida, N., and Sato, K. (2003) Spherical aggregates of beta-amyloid (amylospheroid) show high neurotoxicity and activate tau protein kinase I/glycogen synthase kinase-3beta, Proc. Natl. Acad. Sci. U.S.A. 100, 6370-6375.
21. Andersson, K., Olofsson, A., Nielsen, E. H., Svehag, S. E., and Lundgren, E. (2002) Only amyloidogenic intermediates of transthyretin induce apoptosis, Biochem. Biophys. Res. Commun. 294, 309-314.
22. Reixach, N., Deechongkit, S., Jiang, X., Kelly, J. W., and Buxbaum, J. N. (2004) Tissue damage in the amyloidoses: Transthyretin monomers and nonnative oligomers are the major cytotoxic species in tissue culture, Proc. Natl. Acad. Sci. U.S.A. 101, 2817-2822.
23. De Lucia, R., Mauro, A., Di Scapio, A., Buffo, A., Mortara, R., Orsi, L., and Schiffer, D. (1993) A new mutation on the transthyretin gene (Ser112 to Ile) causes an amyloid neuropathy with severe cardiac impairment, Clin. Neuropathol. 12, S44.
24. Matsubara, K., Mizuguchi, M., and Kawano, K. (2003) Expression of a synthetic gene encoding human transthyretin in Escherichia coli, Protein Expression Purif. 30, 55-61.
25. Hammarstrom, P., Jiang, X., Deechongkit, S., and Kelly, J. W. (2001) Anion shielding of electrostatic repulsions in transthyretin modulates stability and amyloidosis: insight into the chaotrope unfolding dichotomy, Biochemistry 40, 11453-11459.
26. Shinohara, Y., Mizuguchi, M., Matsubara, K., Takeuchi, M., Matsuura, A., Aoki, T., Igarashi, K., Nagadome, H., Terada, Y., and Kawano, K. (2003) Biophysical analyses of the transthyretin variants, Tyr114His and Tyr116Ser, associated with familial amyloidotic polyneuropathy, Biochemistry 42, 15053-15060.
27. Miroy, G. J., Lai, Z., Lashuel, H. A., Peterson, S. A., Strang, C., and Kelly, J. W. (1996) Inhibiting transthyretin amyloid fibril formation via protein stabilization, Proc. Natl. Acad. Sci. U.S.A. 93, 15051-15056.
28. McCafferty, A. C., and Cree, I. A. (1994) Measurement of cell migration stimulated by interleukin 8: use of ATP chemiluminescence, Cytokine 6, 450-453.
29. Sommers, P. B., and Kronman, M. J. (1980) Comparative fluorescence properties of bovine, goat, human and guinea pig alpha lactalbumin. Characterization of the environments of individual tryptophan residues in partially folded conformers, Biophys. Chem. 11, 217-232.
30. Kuwajima, K., Nitta, K., Yoneyama, M., and Sugai, S. (1976) Three-state denaturation of alpha-lactalbumin by guanidine hydrochloride, J. Mol. Biol. 106, 359-373.
31. Santoro, M. M., and Bolen, D. W. (1988) Unfolding free energy changes determined by the linear extrapolation method. 1. Unfolding of phenylmethanesulfonyl alpha-chymotrypsin using different denaturants, Biochemistry 27, 8063-8068.
32. Eftink, M. R., and Ghiron, C. A. (1976) Exposure of tryptophanyl residues in proteins. Quantitative determination by fluorescence quenching studies, Biochemistry 15, 672-680.
33. Eftink, M. R., and Ghiron, C. A. (1981) Fluorescence quenching studies with proteins, Anal. Biochem. 114, 199-227.
34. Semisotnov, G. V., Rodionova, N. A., Razgulyaev, O. I., Uversky, V. N., Gripas', A. F., and Gilmanshin, R. I. (1991). Study of the "molten globule" intermediate state in protein folding by a hydrophobic fluorescent probe, Biopolymers 31, 119-128.
35. Dahlgren, K. N., Manelli, A. M., Stine, W. B., Baker, L. K., Krafft, G. A., and Ladu, M. J. (2002) Oligomeric and fibrillar species of amyloid-beta peptides differentially affect neuronal viability, J. Biol. Chem. 277, 32046-32053.
36. Huang, T. H., Yang, D. S., Plaskos, N. P., Go, S., Yip, C. M., Fraser, P. E., and Chakrabartty, A. (2000) Structural studies of soluble oligomers of the Alzheimer beta-amyloid peptide, J. Mol. Biol. 297, 73-87.
37. Vesenka, J., Guthold, M., Tang, C. L., Keller, D., Delaine, E., and Bustamante, C. (1992) Substrate preparation for reliable imaging of DNA molecules with the scanning force microscope, Ultramicroscopy 42-44, 1243-1249.
38. Sousa, M. M., Cardoso, I., Fernandes, R., Guimaraes, A., and Saraiva, M. J. (2001) Deposition of transthyretin in early stages of familial amyloidotic polyneuropathy: evidence for toxicity of nonfibrillar aggregates, Am. J. Pathol. 159, 1993-2000.
39. Sousa, M. M., Fernandes, R., Palha, J. A., Taboada, A., Vieira, P., and Saraiva, M. J. (2002) Evidence for early cytotoxic aggregates in transgenic mice for human transthyretin Leu55Pro, Am. J. Pathol. 161, 1935-1948.