In vitro studies of amyloid β-protein fibril assembly and toxicity provide clues to the aetiology of Flemish variant (Ala692 → Gly) Alzheimer's disease

Biochemical Journal

Volumn 355, Issue 3, 2001, Pages 869-877

  Walsh, Dominic M ^a   Hartley, Dean M ^a   Condron, Margaret M ^a   Selkoe, Dennis J ^a   Teplow, David B ^a  

^a NONE

Author keywords

Fibrillogenesis; Neurotoxicity

Indexed keywords

AMINO ACIDS; CELLS; OLIGOMERS; TOXICITY;

AETIOLOGY;

PROTEINS;

ALANINE; AMYLOID BETA PROTEIN; AMYLOID PRECURSOR PROTEIN; COMPLEMENTARY DNA; GLYCINE; SYNTHETIC PEPTIDE;

ALZHEIMER DISEASE; AMINO ACID SUBSTITUTION; ANIMAL CELL; ARTICLE; NERVE CELL CULTURE; NERVE CELL NECROSIS; NEUROLOGIC DISEASE; NEUROTOXICITY; NONHUMAN; PEPTIDE ANALYSIS; PRIORITY JOURNAL; RAT; SENILE PLAQUE; SOLUBILITY;

ANIMALIA;

EID: 0035339688     PISSN: 02646021     EISSN: None     Source Type: Journal    
DOI: 10.1042/bj3550869     Document Type: Article

References (51)

1. 1 Iqbal, K. (1991) Prevalence and neurobiology of Alzheimer's disease. In Alzheimer's Disease: Basic Mechanisms, Diagnosis and Therapeutic Strategies (Iqbal, K., McLachian, D. R. C., Winblad, B. and Winsniewski, H. M., eds.), pp, 1-5, John Wiley and Sons, New York
2. 2 Ewbank, D. C. (1999) Deaths attributable to Alzheimer's disease in the United States. Am. J. Pub. Health 89, 90-92
3. 3 Selkoe, D. J. (1991) The molecular pathology of Alzheimer's disease. Neuron 6 487-498
4. 4 Glenner, G. G. and Wong, C. W. (1984) Alzheimer's disease: initial report of the purificatlon and characterization of a novel cerebrovascular amyloid protein. Biochem. Biophys. Res, Commun. 120, 885-890
5. 5 Selkoe, D. J. (1996) Amyloid β-protein and the genetics of Alzheimer's disease. J. Biol. Chem. 271, 18295-18298
6. 6 Selkoe, D. J. (1998) The cell biology of β-amyloid precursor protein and presenilin in Alzheimer's disease. Trends Cell Biol. 8, 447-453
7. 7 Hardy, J. (1997) Amyloid, the presenilins and Alzheimer's disease. Trends Neurosci. 20, 154-159
8. 8 Levy, E., Carman, M. D., Fernandez-Madrid, I. J., Power, M. D., Lieberburg, I., van Duinen, S. G., Bots, G. T. A. M., Luyendljk, W. and Frangione, B. (1990) Mutation of the Alzheimer's disease amyloid gene in hereditary cerebral hemorrhage, Dutch-type, Science 248, 1124-1126
9. 9 Hendriks, L., van Duijn, C. M., Cras, P., Cruts, M., Van Hul, W., van Harskamp, F., Warren, A., McInnis, M. G., Antonarakis, S. E., Martin, J. J. et al. (1992) Presenile dementia and cerebral haemorrhage linked to a mutation at codon 692 of the β-amyloid precursor protein gene. Nat. Genet. 1, 218-221
10. 10 Nilsberth, C., Forsell, C., Axelman, K., Gustafsson, C., Luthman, J., Naslund, J. and Lannfelt, L. (1999) A novel APP mutation (E693G) - the Arctic mutation, causing Alzheimer's disease with vascular symptoms. Soc. Neurosci. Abstr. 25, 297 [120.4]
11. 11 Kamino, K., Orr, H. T., Payami, H., Wijsman, E. M., Alonso, E., Pulst, S. M., Anderson, L., O'dahl, S., Nemens, E., White, J. A. et al. (1992) Linkage and mutational analysis of familial Alzheimer disease kindreds for the APP gene region. Am, J. Hum. Genet. 51, 998-1014
12. 12 Bugiani, O., Padovani, A., Magoni, M., Andora, G., Sgarzi, M., Savolardo, M., Blzzi, A., Giaccone, G., Rossi, G. and Tagllavini, F. (1998) An Italian type of HCHWA. Neurobiol. Aging 19, S238
13. 13 Grabowski, T. J., Cho, H. S., Vonsattel, J. P. G., Rebeck, G. W. and Greenberg, S. M. (2001) A novel APP mutation in an Iowa family with dementia and severe cerebral amyloid angiopathy. Ann. Neurol., In the press
14. 14 Cras, P., van Harskamp, F., Hendriks, L., Ceuterick, C., van Duijn, C. M., Stefanko, S. Z., Hofman, A., Kros, J. M., Van Broeckhoven, C. and Martin, J. J. (1998) Presenile Alzheimer dementia characterized by amyloid angiopathy and large amyloid core type senile plaques in the APP 692Ala → Gly mutation. Acta Neuropathol. 96, 253-260
15. 15 Maat-Schieman, M. L. C., van Duinen, S. G., Bornebroek, M., Haan, J. and Roos, R. A. C. (1996) Hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D). 2. A review of histopathological aspects. Brain Pathol. 6, 115-120
16. 693 → Gln 'Dutch' mutation on the production and stability of amyloid β-protein. Biochem. J. 340, 703-709
17. 17 Wisniewski, T., Ghiso, J. and Frangione, B. (1991) Peptides homologous to the amyloid protein of Alzheimer's disease containing a glutamine for glutamic acid substitution have accelerated amyloid fibril formation. Biochem. Biophys. Res. Commun. 179, 1247-1254
18. 2+ to Gly on the aggregation of a synthetic fragment of the Alzheimer's amyloid β/A4 peptide, Neurosci. Lett. 161, 17-20
19. 19 Fablan, H., Szendrei, G. I., Mantsch, H. H. and Otvos, Jr., L. (1993) Comparative analysis of human and Dutch-type Alzheimer β-amyloid peptides by infrared spectroscopy and circular dichroism. Biochem. Biophys. Res. Commun, 191, 232-239
20. 20 Clements, A., Allsop, D., Walsh, D. M. and Williams, C. H. (1996) Aggregation and metal-binding properties of mutant forms of the amyloid Aβ peptide of Alzheimer's disease. J. Neurochem. 66, 740-747
21. 21 Teplow, D. B., Lomakin, A., Benedek, G. B., Kirschner, D. A. and Walsh, D. M. (1997) Effects of β-protein mutations on amyloid fibril nucleation and elongation. In Alzheimer's Disease: Biology, Diagnosis and Therapeutics (Iqbal, K., Winblad, B., Nishimura, T., Takeda, M. and Wisniewski, H. M., eds.), pp. 311-319, John Wiley and Sons Ltd., Chichester
22. 22 Sian, A. K., Frears, E. R., El-Agnaf, O. M. A., Patel, B. P., Manca, M. F., Siligardi, G., Hussain, R. and Austen, B. M. (2000) Oligomerization of β-amyloid of the Alzheimer's and the Dutch-cerebral-haemorrhage types. Biochem. J. 349, 299-308
23. 23 Fraser, P. E., Nguyen, J. T., Inouye, H., Surewicz, W. K., Selkoe, D. J., Podlisny, M. B. and Kirschner, D. A. (1992) Fibril formation by primate, rodent, and Dutch-hemorrhagic analogues of Alzheimer amyloid β-protein. Biochemistry 31, 10716-10723
24. 24 Van Nostrand, W. E., Melchor, J. P. and Roffini, L. (1998) Pathologic amyloid β-protein cell surface fibril assembly on cultured human cerebrovascular smooth muscle cells. J. Neurochem. 70, 216-223
25. 25 Melchor, J. P., McVoy, L. and Van Nostrand, W. E. (2000) Charge alterations of E22 enhance the pathogenic properties of the amyloid β-protein. J. Neurochem. 74, 2209-2212
26. 26 Roos, R. A., Haan, J. and Van Broeckhoven, C. (1991) Hereditary cerebral hemorrhage with amyloidosis-Dutch type: a congophilic angiopathy. An overview. Ann. N.Y: Acad. Sci. 640, 155-160
27. 27 Walsh, D. M., Lomakin, A., Benedek, G. B., Condron, M. M. and Teplow, D. B. (1997) Amyloid β-protein fibfillogenesis. Detection of a protofibrillar Intermediate. J. Biol. Chem. 272, 22364-22372
28. 28 Larner, A. J. (1999) Hypothesis: amyloid β-peptides truncated at the N-terminus contribute to the pathogenesis of Alzheimer's disease, Neurobiol. Aging 20, 65-69
29. 29 Tekirian, T. L., Yang, A. Y., Glabe, C. and Geddes, J. W. (1999) Toxicity of pyroglutaminated amyloid β-peptides 3(pE)-40 and-42 is similar to that of Aβ 1-40 and -42. J. Neurochem. 73, 1584-1589
30. 30 Pike, C. J., Overman, M. J. and Cotman, C. W. (1995) Amino-terminal deletions enhance aggregation of β-amyloid peptides in vitro, J. Biol. Chem. 270, 23895-23898
31. 31 Haass, C., Hung, A. Y., Selkoe, D. J. and Teplow, D. B. (1994) Mutations associated with a locus for familial Alzheimer's disease result in alternative processing of amyloid β-protein precursor. J. Biol. Chem. 269, 17741-17748
32. 32 Walsh, D. M., Hartley, D. M., Kusumoto, Y., Fezoui, Y., Condron, M. M., Lomakin, A., Benedek, G. B., Selkoe, D. J. and Teplow, D. B. (1999) Amyloid β-protein fibrillogenesis. Structure and biological activity of protofibrillar intermediates. J. Biol. Chem. 274, 25945-25952
33. 33 Ida, N., Hartmann, T., Pantel, J., Schroder, J., Zerfass, R., Forstl, H., Sandbrink, R., Masters, C. L. and Beyreuther, K. (1996) Analysis of heterogeneous βA4 peptides in human cerebrospinal fluid and blood by a newly developed sensitive Western blot assay. J. Biol. Chem. 271, 22908-22914
34. 34 Johnson-Wood, K., Lee, M., Motter, R., Hu, K., Gordon, G., Barbour, R., Khan, K., Gordon, M., Tan, H., Games, D. et al. (1997) Amyloid precursor protein processing and Aβ(42) deposition in a transgenic mouse model of Alzheimer disease. Proc. Natl. Acad. Sci. U.S.A. 94, 1550-1555
35. 2+ accumulation in cortical cell culture correlates with subsequent neuronal degeneration. J. Neurosci. 13, 1993-2000
36. 36 Hartley, D. M., Walsh, D. M., Ye, C. P. P., Diehl, T., Vasquez, S., Vassilev, P. M., Teplow, D. B. and Selkoe, D. J. (1999) Protofibrillar intermediates of amyloid β-protein induce acute electrophysiological changes and progressive neurotoxicity in cortical neurons. J. Neurosci. 19, 8876-8884
37. 37 Burdick, D., Soreghan, B., Kwon, M., Kosmoski, J., Knauer, M., Henschen, A., Yates, J., Cotman, C. and Glabe, C. (1992) Assembly and aggregation properties of synthetic Alzheimer's A4/β amyloid peptide analogs. J. Biol. Chem. 267, 546-554
38. 38 Pike, C. J., Burdick, D., Walencewicz, A. J., Glabe, C. G. and Cotman, C. W. (1993) Neurodegeneration induced by β-amyloid peptides in vitro: The role of peptide assembly state. J. Neurosci. 13, 1676-1687
39. 39 Harper, J. D., Wong, S. S., Lieber, C. M. and Lansbury, P. T. (1997) Observation of metastable Aβ amyloid protofibrils by atomic force microscopy. Chem. Biol. 4, 119-125
40. 40 De Jonghe, C., Zehr, C., Yager, D., Prada, C. M., Younkin, S., Hendriks, L., Van Broeckhoven, C. and Eckman, C. B. (1998) Flemish and Dutch mutations in amyloid β precursor protein have different effects on amyloid β secretion. Neurobiol, Dis. 5, 281-286
41. 41 El-Agnal, O. M. A., Guthrie, D. J. S., Walsh, D. M. and Irvine, G. B. (1998) The influence of the central region containing residues 19-25 on the aggregation properties and secondary structure of Alzheimers β-amyloid peptide. Eur. J. Biochem. 256, 560-569
42. 42 Zhang, S. S., Casey, N. and Lee, J. P. (1998) Residual structure in the Alzheimers-disease peptide - probing the origin of a central hydrophobic cluster, Folding Design 3, 413-422
43. 43 Esler, W. P., Stimson, E. R., Ghilardi, J. R., Lu, Y. A., Felix, A. M., Vinters, H. V., Mantyh, P. W., Lee, J. P. and Magglo, J. E. (1996) Point substitution in the central hydrophobic cluster of a human beta-amyloid congener disrupts peptide folding and abolishes plaque competence. Biochemistry 35, 13914-13921
44. 44 Soreghan, B., Kosmoski, J. and Glabe, C. (1994) Surfactant properties of Alzheimer's Aβ peptides and the mechanism of amyloid aggregation. J. Biol. Chem. 269, 28551-28554
45. 45 Conway, K. A., Lee, S. J., Rochet, J. C., Ding, T. T., Williamson, R. E. and Lansbury, P. T. (2000) Acceleration of oligomerization, not fibrillization, is a shared property of both α-synuclein mutations linked to early-onset Parkinson's disease: Implications for pathogenesis and therapy. Proc. Natl. Acad. Sci. U.S.A. 97, 571-576
46. 46 Harper, J. D. and Lansbury, P. T. Jr. (1997) Models of amyloid seeding in Alzheimer's disease and scrapie: mechanistic truths and physiological consequences of the time-dependent solubility of amyloid proteins. Annu. Rev. Biochem. 66, 385-407
47. 47 Lomakin, A., Teplow, D. B., Klrschner, D. A. and Benedek, G. B. (1997) Kinetic theory of fibrillogenesis of amyloid β-protein. Proc. Natl. Acad. Sci. U.S.A. 94, 7942-7947
48. 48 Kumar-Singh, S., Ceuterick, C., Lubke, U., Merken, M. and Martin, J.-J. (2000) Pathology of Flemish APP692 Alzheimer's disease suggests that core containing plaques are anglocentric. Neurobiol. Aging 21, S66 [297]
49. 1-42 are potent central nervous system neurotoxins. Proc. Natl. Acad. Sci. U.S.A. 95, 6448-6453
50. 50 Kumar-Singh, S., Dewachter, I., Moechars, D., Lubke, U., De Jonghe, C., Ceuterick, C., Checler, F., Naidu, A., Cordell, B., Cras, P. et al. (2000) Behavioral disturbances without amyloid deposits in mice overexpressing human amyloid precursor protein with Flemish (A692G) or Dutch (E693Q) mutation. Neurobiol. Dis. 7, 9-22
51. 51 Wang, Z. Z., Natte, R., Berliner, J. A., van Duinen, S. G. and Vinters, H. V. (2000) Toxicity of Dutch (E22Q) and Flemish (A21G) mutant amyloid β proteins to human cerebral microvessel and aortic smooth muscle cells. Stroke 31, 534-538