Skip to main content
PMC home page
Proceedings of the National Academy of Sciences of the United States of America logoLink to Proceedings of the National Academy of Sciences of the United States of America
. 1993 Jul 15;90(14):6381–6384. doi: 10.1073/pnas.90.14.6381

Alzheimer disease and the prion disorders amyloid beta-protein and prion protein amyloidoses.

D L Price 1, D R Borchelt 1, S S Sisodia 1
PMCID: PMC46935  PMID: 8101988

Abstract

Alzheimer disease and the prion disorders/spongiform encephalopathies share many common features. These chronic, progressive, sometimes familial diseases of the central nervous system are characterized by the presence of different types of amyloid deposits in the brain. This review provides a perspective on these two types of neurodegenerative disorders.

Full text

PDF
6381

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Barrow C. J., Zagorski M. G. Solution structures of beta peptide and its constituent fragments: relation to amyloid deposition. Science. 1991 Jul 12;253(5016):179–182. doi: 10.1126/science.1853202. [DOI] [PubMed] [Google Scholar]
  2. Borchelt D. R., Taraboulos A., Prusiner S. B. Evidence for synthesis of scrapie prion proteins in the endocytic pathway. J Biol Chem. 1992 Aug 15;267(23):16188–16199. [PubMed] [Google Scholar]
  3. Braak H., Braak E. Alzheimer's disease affects limbic nuclei of the thalamus. Acta Neuropathol. 1991;81(3):261–268. doi: 10.1007/BF00305867. [DOI] [PubMed] [Google Scholar]
  4. Brown P., Goldfarb L. G., Kovanen J., Haltia M., Cathala F., Sulima M., Gibbs C. J., Jr, Gajdusek D. C. Phenotypic characteristics of familial Creutzfeldt-Jakob disease associated with the codon 178Asn PRNP mutation. Ann Neurol. 1992 Mar;31(3):282–285. doi: 10.1002/ana.410310309. [DOI] [PubMed] [Google Scholar]
  5. Burdick D., Soreghan B., Kwon M., Kosmoski J., Knauer M., Henschen A., Yates J., Cotman C., Glabe C. Assembly and aggregation properties of synthetic Alzheimer's A4/beta amyloid peptide analogs. J Biol Chem. 1992 Jan 5;267(1):546–554. [PubMed] [Google Scholar]
  6. Busciglio J., Gabuzda D. H., Matsudaira P., Yankner B. A. Generation of beta-amyloid in the secretory pathway in neuronal and nonneuronal cells. Proc Natl Acad Sci U S A. 1993 Mar 1;90(5):2092–2096. doi: 10.1073/pnas.90.5.2092. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Buxbaum J. D., Gandy S. E., Cicchetti P., Ehrlich M. E., Czernik A. J., Fracasso R. P., Ramabhadran T. V., Unterbeck A. J., Greengard P. Processing of Alzheimer beta/A4 amyloid precursor protein: modulation by agents that regulate protein phosphorylation. Proc Natl Acad Sci U S A. 1990 Aug;87(15):6003–6006. doi: 10.1073/pnas.87.15.6003. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Büeler H., Fischer M., Lang Y., Bluethmann H., Lipp H. P., DeArmond S. J., Prusiner S. B., Aguet M., Weissmann C. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature. 1992 Apr 16;356(6370):577–582. doi: 10.1038/356577a0. [DOI] [PubMed] [Google Scholar]
  9. Cai X. D., Golde T. E., Younkin S. G. Release of excess amyloid beta protein from a mutant amyloid beta protein precursor. Science. 1993 Jan 22;259(5094):514–516. doi: 10.1126/science.8424174. [DOI] [PubMed] [Google Scholar]
  10. Castaño E. M., Ghiso J., Prelli F., Gorevic P. D., Migheli A., Frangione B. In vitro formation of amyloid fibrils from two synthetic peptides of different lengths homologous to Alzheimer's disease beta-protein. Biochem Biophys Res Commun. 1986 Dec 15;141(2):782–789. doi: 10.1016/s0006-291x(86)80241-8. [DOI] [PubMed] [Google Scholar]
  11. Chartier-Harlin M. C., Crawford F., Houlden H., Warren A., Hughes D., Fidani L., Goate A., Rossor M., Roques P., Hardy J. Early-onset Alzheimer's disease caused by mutations at codon 717 of the beta-amyloid precursor protein gene. Nature. 1991 Oct 31;353(6347):844–846. doi: 10.1038/353844a0. [DOI] [PubMed] [Google Scholar]
  12. Citron M., Oltersdorf T., Haass C., McConlogue L., Hung A. Y., Seubert P., Vigo-Pelfrey C., Lieberburg I., Selkoe D. J. Mutation of the beta-amyloid precursor protein in familial Alzheimer's disease increases beta-protein production. Nature. 1992 Dec 17;360(6405):672–674. doi: 10.1038/360672a0. [DOI] [PubMed] [Google Scholar]
  13. Cole G. M., Huynh T. V., Saitoh T. Evidence for lysosomal processing of amyloid beta-protein precursor in cultured cells. Neurochem Res. 1989 Oct;14(10):933–939. doi: 10.1007/BF00965926. [DOI] [PubMed] [Google Scholar]
  14. Cork L. C., Masters C., Beyreuther K., Price D. L. Development of senile plaques. Relationships of neuronal abnormalities and amyloid deposits. Am J Pathol. 1990 Dec;137(6):1383–1392. [PMC free article] [PubMed] [Google Scholar]
  15. De Souza E. B., Whitehouse P. J., Kuhar M. J., Price D. L., Vale W. W. Reciprocal changes in corticotropin-releasing factor (CRF)-like immunoreactivity and CRF receptors in cerebral cortex of Alzheimer's disease. Nature. 1986 Feb 13;319(6054):593–595. doi: 10.1038/319593a0. [DOI] [PubMed] [Google Scholar]
  16. Esch F. S., Keim P. S., Beattie E. C., Blacher R. W., Culwell A. R., Oltersdorf T., McClure D., Ward P. J. Cleavage of amyloid beta peptide during constitutive processing of its precursor. Science. 1990 Jun 1;248(4959):1122–1124. doi: 10.1126/science.2111583. [DOI] [PubMed] [Google Scholar]
  17. Evans D. A., Funkenstein H. H., Albert M. S., Scherr P. A., Cook N. R., Chown M. J., Hebert L. E., Hennekens C. H., Taylor J. O. Prevalence of Alzheimer's disease in a community population of older persons. Higher than previously reported. JAMA. 1989 Nov 10;262(18):2551–2556. [PubMed] [Google Scholar]
  18. Gajdusek D. C., Gibbs C. J., Alpers M. Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature. 1966 Feb 19;209(5025):794–796. doi: 10.1038/209794a0. [DOI] [PubMed] [Google Scholar]
  19. Gasset M., Baldwin M. A., Lloyd D. H., Gabriel J. M., Holtzman D. M., Cohen F., Fletterick R., Prusiner S. B. Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid. Proc Natl Acad Sci U S A. 1992 Nov 15;89(22):10940–10944. doi: 10.1073/pnas.89.22.10940. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Ghetti B., Tagliavini F., Masters C. L., Beyreuther K., Giaccone G., Verga L., Farlow M. R., Conneally P. M., Dlouhy S. R., Azzarelli B. Gerstmann-Sträussler-Scheinker disease. II. Neurofibrillary tangles and plaques with PrP-amyloid coexist in an affected family. Neurology. 1989 Nov;39(11):1453–1461. doi: 10.1212/wnl.39.11.1453. [DOI] [PubMed] [Google Scholar]
  21. Gibbs C. J., Jr, Gajdusek D. C., Asher D. M., Alpers M. P., Beck E., Daniel P. M., Matthews W. B. Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to the chimpanzee. Science. 1968 Jul 26;161(3839):388–389. doi: 10.1126/science.161.3839.388. [DOI] [PubMed] [Google Scholar]
  22. Glenner G. G., Wong C. W. Alzheimer's disease: initial report of the purification and characterization of a novel cerebrovascular amyloid protein. Biochem Biophys Res Commun. 1984 May 16;120(3):885–890. doi: 10.1016/s0006-291x(84)80190-4. [DOI] [PubMed] [Google Scholar]
  23. Goate A., Chartier-Harlin M. C., Mullan M., Brown J., Crawford F., Fidani L., Giuffra L., Haynes A., Irving N., James L. Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease. Nature. 1991 Feb 21;349(6311):704–706. doi: 10.1038/349704a0. [DOI] [PubMed] [Google Scholar]
  24. Goedert M., Jakes R., Crowther R. A., Six J., Lübke U., Vandermeeren M., Cras P., Trojanowski J. Q., Lee V. M. The abnormal phosphorylation of tau protein at Ser-202 in Alzheimer disease recapitulates phosphorylation during development. Proc Natl Acad Sci U S A. 1993 Jun 1;90(11):5066–5070. doi: 10.1073/pnas.90.11.5066. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Golde T. E., Estus S., Younkin L. H., Selkoe D. J., Younkin S. G. Processing of the amyloid protein precursor to potentially amyloidogenic derivatives. Science. 1992 Feb 7;255(5045):728–730. doi: 10.1126/science.1738847. [DOI] [PubMed] [Google Scholar]
  26. Goldfarb L. G., Brown P., Haltia M., Ghiso J., Frangione B., Gajdusek D. C. Synthetic peptides corresponding to different mutated regions of the amyloid gene in familial Creutzfeldt-Jakob disease show enhanced in vitro formation of morphologically different amyloid fibrils. Proc Natl Acad Sci U S A. 1993 May 15;90(10):4451–4454. doi: 10.1073/pnas.90.10.4451. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Goldfarb L. G., Brown P., Vrbovská A., Baron H., McCombie W. R., Cathala F., Gibbs C. J., Jr, Gajdusek D. C. An insert mutation in the chromosome 20 amyloid precursor gene in a Gerstmann-Sträussler-Scheinker family. J Neurol Sci. 1992 Sep;111(2):189–194. doi: 10.1016/0022-510x(92)90067-u. [DOI] [PubMed] [Google Scholar]
  28. Goldfarb L. G., Haltia M., Brown P., Nieto A., Kovanen J., McCombie W. R., Trapp S., Gajdusek D. C. New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt-Jakob kindred. Lancet. 1991 Feb 16;337(8738):425–425. doi: 10.1016/0140-6736(91)91198-4. [DOI] [PubMed] [Google Scholar]
  29. Goldfarb L. G., Mitrová E., Brown P., Toh B. K., Gajdusek D. C. Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia. Lancet. 1990 Aug 25;336(8713):514–515. doi: 10.1016/0140-6736(90)92073-q. [DOI] [PubMed] [Google Scholar]
  30. Goldgaber D., Goldfarb L. G., Brown P., Asher D. M., Brown W. T., Lin S., Teener J. W., Feinstone S. M., Rubenstein R., Kascsak R. J. Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome. Exp Neurol. 1989 Nov;106(2):204–206. doi: 10.1016/0014-4886(89)90095-2. [DOI] [PubMed] [Google Scholar]
  31. Greenberg S. G., Davies P. A preparation of Alzheimer paired helical filaments that displays distinct tau proteins by polyacrylamide gel electrophoresis. Proc Natl Acad Sci U S A. 1990 Aug;87(15):5827–5831. doi: 10.1073/pnas.87.15.5827. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. Grundke-Iqbal I., Iqbal K., Quinlan M., Tung Y. C., Zaidi M. S., Wisniewski H. M. Microtubule-associated protein tau. A component of Alzheimer paired helical filaments. J Biol Chem. 1986 May 5;261(13):6084–6089. [PubMed] [Google Scholar]
  33. Haass C., Koo E. H., Mellon A., Hung A. Y., Selkoe D. J. Targeting of cell-surface beta-amyloid precursor protein to lysosomes: alternative processing into amyloid-bearing fragments. Nature. 1992 Jun 11;357(6378):500–503. doi: 10.1038/357500a0. [DOI] [PubMed] [Google Scholar]
  34. Haass C., Schlossmacher M. G., Hung A. Y., Vigo-Pelfrey C., Mellon A., Ostaszewski B. L., Lieberburg I., Koo E. H., Schenk D., Teplow D. B. Amyloid beta-peptide is produced by cultured cells during normal metabolism. Nature. 1992 Sep 24;359(6393):322–325. doi: 10.1038/359322a0. [DOI] [PubMed] [Google Scholar]
  35. 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. Presenile dementia and cerebral haemorrhage linked to a mutation at codon 692 of the beta-amyloid precursor protein gene. Nat Genet. 1992 Jun;1(3):218–221. doi: 10.1038/ng0692-218. [DOI] [PubMed] [Google Scholar]
  36. Hsiao K. K., Cass C., Schellenberg G. D., Bird T., Devine-Gage E., Wisniewski H., Prusiner S. B. A prion protein variant in a family with the telencephalic form of Gerstmann-Sträussler-Scheinker syndrome. Neurology. 1991 May;41(5):681–684. doi: 10.1212/wnl.41.5.681. [DOI] [PubMed] [Google Scholar]
  37. Hsiao K. K., Scott M., Foster D., Groth D. F., DeArmond S. J., Prusiner S. B. Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science. 1990 Dec 14;250(4987):1587–1590. doi: 10.1126/science.1980379. [DOI] [PubMed] [Google Scholar]
  38. Hsiao K., Baker H. F., Crow T. J., Poulter M., Owen F., Terwilliger J. D., Westaway D., Ott J., Prusiner S. B. Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Nature. 1989 Mar 23;338(6213):342–345. doi: 10.1038/338342a0. [DOI] [PubMed] [Google Scholar]
  39. Hsiao K., Dlouhy S. R., Farlow M. R., Cass C., Da Costa M., Conneally P. M., Hodes M. E., Ghetti B., Prusiner S. B. Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles. Nat Genet. 1992 Apr;1(1):68–71. doi: 10.1038/ng0492-68. [DOI] [PubMed] [Google Scholar]
  40. Hsiao K., Meiner Z., Kahana E., Cass C., Kahana I., Avrahami D., Scarlato G., Abramsky O., Prusiner S. B., Gabizon R. Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. N Engl J Med. 1991 Apr 18;324(16):1091–1097. doi: 10.1056/NEJM199104183241604. [DOI] [PubMed] [Google Scholar]
  41. Hyman B. T., Van Hoesen G. W., Kromer L. J., Damasio A. R. Perforant pathway changes and the memory impairment of Alzheimer's disease. Ann Neurol. 1986 Oct;20(4):472–481. doi: 10.1002/ana.410200406. [DOI] [PubMed] [Google Scholar]
  42. Iverfeldt K., Walaas S. I., Greengard P. Altered processing of Alzheimer amyloid precursor protein in response to neuronal degeneration. Proc Natl Acad Sci U S A. 1993 May 1;90(9):4146–4150. doi: 10.1073/pnas.90.9.4146. [DOI] [PMC free article] [PubMed] [Google Scholar]
  43. Kammesheidt A., Boyce F. M., Spanoyannis A. F., Cummings B. J., Ortegón M., Cotman C., Vaught J. L., Neve R. L. Deposition of beta/A4 immunoreactivity and neuronal pathology in transgenic mice expressing the carboxyl-terminal fragment of the Alzheimer amyloid precursor in the brain. Proc Natl Acad Sci U S A. 1992 Nov 15;89(22):10857–10861. doi: 10.1073/pnas.89.22.10857. [DOI] [PMC free article] [PubMed] [Google Scholar]
  44. Kang J., Lemaire H. G., Unterbeck A., Salbaum J. M., Masters C. L., Grzeschik K. H., Multhaup G., Beyreuther K., Müller-Hill B. The precursor of Alzheimer's disease amyloid A4 protein resembles a cell-surface receptor. Nature. 1987 Feb 19;325(6106):733–736. doi: 10.1038/325733a0. [DOI] [PubMed] [Google Scholar]
  45. Kawabata S., Higgins G. A., Gordon J. W. Amyloid plaques, neurofibrillary tangles and neuronal loss in brains of transgenic mice overexpressing a C-terminal fragment of human amyloid precursor protein. Nature. 1991 Dec 12;354(6353):476–478. doi: 10.1038/354476a0. [DOI] [PubMed] [Google Scholar]
  46. Kirschner D. A., Inouye H., Duffy L. K., Sinclair A., Lind M., Selkoe D. J. Synthetic peptide homologous to beta protein from Alzheimer disease forms amyloid-like fibrils in vitro. Proc Natl Acad Sci U S A. 1987 Oct;84(19):6953–6957. doi: 10.1073/pnas.84.19.6953. [DOI] [PMC free article] [PubMed] [Google Scholar]
  47. Kitamoto T., Tateishi J., Tashima T., Takeshita I., Barry R. A., DeArmond S. J., Prusiner S. B. Amyloid plaques in Creutzfeldt-Jakob disease stain with prion protein antibodies. Ann Neurol. 1986 Aug;20(2):204–208. doi: 10.1002/ana.410200205. [DOI] [PubMed] [Google Scholar]
  48. Koo E. H., Park L., Selkoe D. J. Amyloid beta-protein as a substrate interacts with extracellular matrix to promote neurite outgrowth. Proc Natl Acad Sci U S A. 1993 May 15;90(10):4748–4752. doi: 10.1073/pnas.90.10.4748. [DOI] [PMC free article] [PubMed] [Google Scholar]
  49. Koo E. H., Sisodia S. S., Archer D. R., Martin L. J., Weidemann A., Beyreuther K., Fischer P., Masters C. L., Price D. L. Precursor of amyloid protein in Alzheimer disease undergoes fast anterograde axonal transport. Proc Natl Acad Sci U S A. 1990 Feb;87(4):1561–1565. doi: 10.1073/pnas.87.4.1561. [DOI] [PMC free article] [PubMed] [Google Scholar]
  50. Koo E. H., Sisodia S. S., Cork L. C., Unterbeck A., Bayney R. M., Price D. L. Differential expression of amyloid precursor protein mRNAs in cases of Alzheimer's disease and in aged nonhuman primates. Neuron. 1990 Jan;4(1):97–104. doi: 10.1016/0896-6273(90)90446-m. [DOI] [PubMed] [Google Scholar]
  51. Kosik K. S., Orecchio L. D., Binder L., Trojanowski J. Q., Lee V. M., Lee G. Epitopes that span the tau molecule are shared with paired helical filaments. Neuron. 1988 Nov;1(9):817–825. doi: 10.1016/0896-6273(88)90129-8. [DOI] [PubMed] [Google Scholar]
  52. Lee V. M., Balin B. J., Otvos L., Jr, Trojanowski J. Q. A68: a major subunit of paired helical filaments and derivatized forms of normal Tau. Science. 1991 Feb 8;251(4994):675–678. doi: 10.1126/science.1899488. [DOI] [PubMed] [Google Scholar]
  53. Martin L. J., Sisodia S. S., Koo E. H., Cork L. C., Dellovade T. L., Weidemann A., Beyreuther K., Masters C., Price D. L. Amyloid precursor protein in aged nonhuman primates. Proc Natl Acad Sci U S A. 1991 Feb 15;88(4):1461–1465. doi: 10.1073/pnas.88.4.1461. [DOI] [PMC free article] [PubMed] [Google Scholar]
  54. Masliah E., Mallory M., Hansen L., Alford M., Albright T., DeTeresa R., Terry R., Baudier J., Saitoh T. Patterns of aberrant sprouting in Alzheimer's disease. Neuron. 1991 May;6(5):729–739. doi: 10.1016/0896-6273(91)90170-5. [DOI] [PubMed] [Google Scholar]
  55. Masters C. L., Multhaup G., Simms G., Pottgiesser J., Martins R. N., Beyreuther K. Neuronal origin of a cerebral amyloid: neurofibrillary tangles of Alzheimer's disease contain the same protein as the amyloid of plaque cores and blood vessels. EMBO J. 1985 Nov;4(11):2757–2763. doi: 10.1002/j.1460-2075.1985.tb04000.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  56. Masters C. L., Simms G., Weinman N. A., Multhaup G., McDonald B. L., Beyreuther K. Amyloid plaque core protein in Alzheimer disease and Down syndrome. Proc Natl Acad Sci U S A. 1985 Jun;82(12):4245–4249. doi: 10.1073/pnas.82.12.4245. [DOI] [PMC free article] [PubMed] [Google Scholar]
  57. McKhann G., Drachman D., Folstein M., Katzman R., Price D., Stadlan E. M. Clinical diagnosis of Alzheimer's disease: report of the NINCDS-ADRDA Work Group under the auspices of Department of Health and Human Services Task Force on Alzheimer's Disease. Neurology. 1984 Jul;34(7):939–944. doi: 10.1212/wnl.34.7.939. [DOI] [PubMed] [Google Scholar]
  58. McKinley M. P., Meyer R. K., Kenaga L., Rahbar F., Cotter R., Serban A., Prusiner S. B. Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis. J Virol. 1991 Mar;65(3):1340–1351. doi: 10.1128/jvi.65.3.1340-1351.1991. [DOI] [PMC free article] [PubMed] [Google Scholar]
  59. Medori R., Tritschler H. J., LeBlanc A., Villare F., Manetto V., Chen H. Y., Xue R., Leal S., Montagna P., Cortelli P. Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. N Engl J Med. 1992 Feb 13;326(7):444–449. doi: 10.1056/NEJM199202133260704. [DOI] [PMC free article] [PubMed] [Google Scholar]
  60. Murrell J., Farlow M., Ghetti B., Benson M. D. A mutation in the amyloid precursor protein associated with hereditary Alzheimer's disease. Science. 1991 Oct 4;254(5028):97–99. doi: 10.1126/science.1925564. [DOI] [PubMed] [Google Scholar]
  61. Naruse S., Igarashi S., Kobayashi H., Aoki K., Inuzuka T., Kaneko K., Shimizu T., Iihara K., Kojima T., Miyatake T. Mis-sense mutation Val----Ile in exon 17 of amyloid precursor protein gene in Japanese familial Alzheimer's disease. Lancet. 1991 Apr 20;337(8747):978–979. doi: 10.1016/0140-6736(91)91612-x. [DOI] [PubMed] [Google Scholar]
  62. Nishimoto I., Okamoto T., Matsuura Y., Takahashi S., Okamoto T., Murayama Y., Ogata E. Alzheimer amyloid protein precursor complexes with brain GTP-binding protein G(o) Nature. 1993 Mar 4;362(6415):75–79. doi: 10.1038/362075a0. [DOI] [PubMed] [Google Scholar]
  63. Nitsch R. M., Farber S. A., Growdon J. H., Wurtman R. J. Release of amyloid beta-protein precursor derivatives by electrical depolarization of rat hippocampal slices. Proc Natl Acad Sci U S A. 1993 Jun 1;90(11):5191–5193. doi: 10.1073/pnas.90.11.5191. [DOI] [PMC free article] [PubMed] [Google Scholar]
  64. Nitsch R. M., Slack B. E., Wurtman R. J., Growdon J. H. Release of Alzheimer amyloid precursor derivatives stimulated by activation of muscarinic acetylcholine receptors. Science. 1992 Oct 9;258(5080):304–307. doi: 10.1126/science.1411529. [DOI] [PubMed] [Google Scholar]
  65. Price D. L., Borchelt D. R., Walker L. C., Sisodia S. S. Toxicity of synthetic A beta peptides and modeling of Alzheimer's disease. Neurobiol Aging. 1992 Sep-Oct;13(5):623–625. doi: 10.1016/0197-4580(92)90069-a. [DOI] [PubMed] [Google Scholar]
  66. Price D. L., Martin L. J., Clatterbuck R. E., Koliatsos V. E., Sisodia S. S., Walker L. C., Cork L. C. Neuronal degeneration in human diseases and animal models. J Neurobiol. 1992 Nov;23(9):1277–1294. doi: 10.1002/neu.480230916. [DOI] [PubMed] [Google Scholar]
  67. Price D. L., Walker L. C., Martin L. J., Sisodia S. S. Amyloidosis in aging and Alzheimer's disease. Am J Pathol. 1992 Oct;141(4):767–772. [PMC free article] [PubMed] [Google Scholar]
  68. Probst A., Langui D., Ipsen S., Robakis N., Ulrich J. Deposition of beta/A4 protein along neuronal plasma membranes in diffuse senile plaques. Acta Neuropathol. 1991;83(1):21–29. doi: 10.1007/BF00294426. [DOI] [PubMed] [Google Scholar]
  69. Prusiner S. B., DeArmond S. J. Molecular biology and pathology of scrapie and the prion diseases of humans. Brain Pathol. 1991 Jul;1(4):297–310. doi: 10.1111/j.1750-3639.1991.tb00673.x. [DOI] [PubMed] [Google Scholar]
  70. Prusiner S. B. Molecular biology of prion diseases. Science. 1991 Jun 14;252(5012):1515–1522. doi: 10.1126/science.1675487. [DOI] [PubMed] [Google Scholar]
  71. Prusiner S. B., Scott M., Foster D., Pan K. M., Groth D., Mirenda C., Torchia M., Yang S. L., Serban D., Carlson G. A. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell. 1990 Nov 16;63(4):673–686. doi: 10.1016/0092-8674(90)90134-z. [DOI] [PubMed] [Google Scholar]
  72. Quon D., Wang Y., Catalano R., Scardina J. M., Murakami K., Cordell B. Formation of beta-amyloid protein deposits in brains of transgenic mice. Nature. 1991 Jul 18;352(6332):239–241. doi: 10.1038/352239a0. [DOI] [PubMed] [Google Scholar]
  73. Rumble B., Retallack R., Hilbich C., Simms G., Multhaup G., Martins R., Hockey A., Montgomery P., Beyreuther K., Masters C. L. Amyloid A4 protein and its precursor in Down's syndrome and Alzheimer's disease. N Engl J Med. 1989 Jun 1;320(22):1446–1452. doi: 10.1056/NEJM198906013202203. [DOI] [PubMed] [Google Scholar]
  74. Schellenberg G. D., Bird T. D., Wijsman E. M., Orr H. T., Anderson L., Nemens E., White J. A., Bonnycastle L., Weber J. L., Alonso M. E. Genetic linkage evidence for a familial Alzheimer's disease locus on chromosome 14. Science. 1992 Oct 23;258(5082):668–671. doi: 10.1126/science.1411576. [DOI] [PubMed] [Google Scholar]
  75. Schellenberg G. D., Boehnke M., Wijsman E. M., Moore D. K., Martin G. M., Bird T. D. Genetic association and linkage analysis of the apolipoprotein CII locus and familial Alzheimer's disease. Ann Neurol. 1992 Feb;31(2):223–227. doi: 10.1002/ana.410310214. [DOI] [PubMed] [Google Scholar]
  76. Schubert D. The possible role of adhesion in synaptic modification. Trends Neurosci. 1991 Apr;14(4):127–130. doi: 10.1016/0166-2236(91)90078-9. [DOI] [PubMed] [Google Scholar]
  77. Selkoe D. J., Bell D. S., Podlisny M. B., Price D. L., Cork L. C. Conservation of brain amyloid proteins in aged mammals and humans with Alzheimer's disease. Science. 1987 Feb 20;235(4791):873–877. doi: 10.1126/science.3544219. [DOI] [PubMed] [Google Scholar]
  78. Selkoe D. J. The molecular pathology of Alzheimer's disease. Neuron. 1991 Apr;6(4):487–498. doi: 10.1016/0896-6273(91)90052-2. [DOI] [PubMed] [Google Scholar]
  79. Seubert P., Vigo-Pelfrey C., Esch F., Lee M., Dovey H., Davis D., Sinha S., Schlossmacher M., Whaley J., Swindlehurst C. Isolation and quantification of soluble Alzheimer's beta-peptide from biological fluids. Nature. 1992 Sep 24;359(6393):325–327. doi: 10.1038/359325a0. [DOI] [PubMed] [Google Scholar]
  80. Shoji M., Golde T. E., Ghiso J., Cheung T. T., Estus S., Shaffer L. M., Cai X. D., McKay D. M., Tintner R., Frangione B. Production of the Alzheimer amyloid beta protein by normal proteolytic processing. Science. 1992 Oct 2;258(5079):126–129. doi: 10.1126/science.1439760. [DOI] [PubMed] [Google Scholar]
  81. Sisodia S. S. Beta-amyloid precursor protein cleavage by a membrane-bound protease. Proc Natl Acad Sci U S A. 1992 Jul 1;89(13):6075–6079. doi: 10.1073/pnas.89.13.6075. [DOI] [PMC free article] [PubMed] [Google Scholar]
  82. Sisodia S. S., Koo E. H., Beyreuther K., Unterbeck A., Price D. L. Evidence that beta-amyloid protein in Alzheimer's disease is not derived by normal processing. Science. 1990 Apr 27;248(4954):492–495. doi: 10.1126/science.1691865. [DOI] [PubMed] [Google Scholar]
  83. Sisodia S. S., Price D. L. Amyloidogenesis in Alzheimer's disease: basic biology and animal models. Curr Opin Neurobiol. 1992 Oct;2(5):648–652. doi: 10.1016/0959-4388(92)90033-h. [DOI] [PubMed] [Google Scholar]
  84. Stahl N., Baldwin M. A., Teplow D. B., Hood L., Gibson B. W., Burlingame A. L., Prusiner S. B. Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing. Biochemistry. 1993 Mar 2;32(8):1991–2002. doi: 10.1021/bi00059a016. [DOI] [PubMed] [Google Scholar]
  85. Strittmatter W. J., Saunders A. M., Schmechel D., Pericak-Vance M., Enghild J., Salvesen G. S., Roses A. D. Apolipoprotein E: high-avidity binding to beta-amyloid and increased frequency of type 4 allele in late-onset familial Alzheimer disease. Proc Natl Acad Sci U S A. 1993 Mar 1;90(5):1977–1981. doi: 10.1073/pnas.90.5.1977. [DOI] [PMC free article] [PubMed] [Google Scholar]
  86. Terry R. D., Masliah E., Salmon D. P., Butters N., DeTeresa R., Hill R., Hansen L. A., Katzman R. Physical basis of cognitive alterations in Alzheimer's disease: synapse loss is the major correlate of cognitive impairment. Ann Neurol. 1991 Oct;30(4):572–580. doi: 10.1002/ana.410300410. [DOI] [PubMed] [Google Scholar]
  87. Wang R., Meschia J. F., Cotter R. J., Sisodia S. S. Secretion of the beta/A4 amyloid precursor protein. Identification of a cleavage site in cultured mammalian cells. J Biol Chem. 1991 Sep 5;266(25):16960–16964. [PubMed] [Google Scholar]
  88. Weidemann A., König G., Bunke D., Fischer P., Salbaum J. M., Masters C. L., Beyreuther K. Identification, biogenesis, and localization of precursors of Alzheimer's disease A4 amyloid protein. Cell. 1989 Apr 7;57(1):115–126. doi: 10.1016/0092-8674(89)90177-3. [DOI] [PubMed] [Google Scholar]
  89. Whitehouse P. J., Price D. L., Struble R. G., Clark A. W., Coyle J. T., Delon M. R. Alzheimer's disease and senile dementia: loss of neurons in the basal forebrain. Science. 1982 Mar 5;215(4537):1237–1239. doi: 10.1126/science.7058341. [DOI] [PubMed] [Google Scholar]
  90. Wirak D. O., Bayney R., Ramabhadran T. V., Fracasso R. P., Hart J. T., Hauer P. E., Hsiau P., Pekar S. K., Scangos G. A., Trapp B. D. Deposits of amyloid beta protein in the central nervous system of transgenic mice. Science. 1991 Jul 19;253(5017):323–325. doi: 10.1126/science.1857970. [DOI] [PubMed] [Google Scholar]

Articles from Proceedings of the National Academy of Sciences of the United States of America are provided here courtesy of National Academy of Sciences

RESOURCES