@article{17d7dc3fa4694e2db4f3d776b7707856,
title = "Amyloid-β-protein isoforms in brain of subjects with PS1-linked, βAPP-linked and sporadic Alzheimer disease",
abstract = "To determine whether similar abnormalities of various soluble full-length and N-terminal truncated Aβ peptides occur in postmortem cerebral cortex of affected PS1 mutation carriers, we examined the amounts of two amyloid species ending at residue 40 or at residues 42(43) using sandwich ELISA systems. Our results indicate that PS1 mutations effect a dramatic accumulation in brain of the highly insoluble potentially neurotoxic long-tailed isoforms of the Aβ peptide such as Aβ1-42(43) and Aβx-42(43). This enhancing effect of PS1 mutation on Aβx-42(43) deposition was highly similar to that of a βAPP mutation (Val717Ile) but the effects on Aβx-40 production were significantly different between these two causal genes. In contrast to previous studies of soluble Aβ in plasma and in supernatants from cultured fibroblasts of subjects with PS1 mutations, our studies also show that there is an increase in insoluble Aβx-40 peptides in brain of subjects with PS1 mutations.",
keywords = "APP717, Alzheimer's disease, Amyloid deposition, Insoluble, Presenilin-1",
author = "Akira Tamaoka and Fraser, {Paul E.} and Kazuhiro Ishii and Naruhiko Sahara and Kazuharu Ozawa and Masaki Ikeda and Saunders, {Ann M.} and Yasuko Komatsuzaki and Robin Sherrington and Georges Levesque and Gang Yu and Ekaterina Rogaeva and Shin'Ichi Shoji and Nee, {Linda E.} and Pollen, {Daniel A.} and Lydia Hendriks and Martin, {Jean J.} and {Van Broeckhoven}, Christine and Roses, {Allen D.} and Farrer, {Lindsay A.} and {St. George-Hyslop}, {Peter H.} and Hiroshi Mori",
note = "Funding Information: This work was supported in part by Grants-in-Aid for Scientific Research on Priority Areas from the Ministry of Education, Science and Culture, Japan (to H.M.) and Health and Welfare, Japan (to A.T. and H.M.); by NIH grant AG09029 (to L.A.F.), and by the Fund for Scientific Research-Flanders (Belgium) (FWO), the Flemish Biotechnology Programme (COT-004), the DWTC InterUniversity Attraction Poles (IUAP) and the European Union BIOTECH grant CT96 (to C.V.B and J.-J.M.), and by grants from the Medical Research Council of Canada, the Alzheimer Association of Ontario, EJLB Foundation, Howard Hughes Medical Research Foundation and the Canadian Genetic Disease Network (to P.H. St.G.-H.). Thanks are also due to the Bryan Alzheimer's Disease Research Center funded by NIH AG05128 and Glaxo Wellcome. Some of the control brains used in this study were also obtained from the Canadian Brain Tissue Bank.",
year = "1998",
month = may,
doi = "10.1016/S0169-328X(98)00044-8",
language = "English (US)",
volume = "56",
pages = "178--185",
journal = "Brain Research",
issn = "0006-8993",
publisher = "Elsevier",
number = "1-2",
}