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@ARTICLE{Nussbaum:136529,
author = {Nussbaum, Justin M and Schilling, Stephan and Cynis, Holger
and Silva, Antonia and Swanson, Eric and Wangsanut, Tanaporn
and Tayler, Kaycie and Wiltgen, Brian and Hatami, Asa and
Rönicke, Raik and Reymann, Klaus and Hutter-Paier, Birgit
and Alexandru, Anca and Jagla, Wolfgang and Graubner, Sigrid
and Glabe, Charles G and Demuth, Hans-Ulrich and Bloom,
George S},
title = {{P}rion-like behaviour and tau-dependent cytotoxicity of
pyroglutamylated amyloid-β.},
journal = {Nature},
volume = {485},
number = {7400},
issn = {0028-0836},
address = {London [u.a.]},
publisher = {Nature Publ. Group65848},
reportid = {DZNE-2020-02851},
pages = {651-655},
year = {2012},
abstract = {Extracellular plaques of amyloid-β and intraneuronal
neurofibrillary tangles made from tau are the
histopathological signatures of Alzheimer's disease. Plaques
comprise amyloid-β fibrils that assemble from monomeric and
oligomeric intermediates, and are prognostic indicators of
Alzheimer's disease. Despite the importance of plaques to
Alzheimer's disease, oligomers are considered to be the
principal toxic forms of amyloid-β. Interestingly, many
adverse responses to amyloid-β, such as cytotoxicity,
microtubule loss, impaired memory and learning, and neuritic
degeneration, are greatly amplified by tau expression.
Amino-terminally truncated, pyroglutamylated (pE) forms of
amyloid-β are strongly associated with Alzheimer's disease,
are more toxic than amyloid-β, residues 1-42 (Aβ(1-42))
and Aβ(1-40), and have been proposed as initiators of
Alzheimer's disease pathogenesis. Here we report a mechanism
by which pE-Aβ may trigger Alzheimer's disease.
Aβ(3(pE)-42) co-oligomerizes with excess Aβ(1-42) to form
metastable low-n oligomers (LNOs) that are structurally
distinct and far more cytotoxic to cultured neurons than
comparable LNOs made from Aβ(1-42) alone. Tau is required
for cytotoxicity, and LNOs comprising $5\%$ Aβ(3(pE)-42)
plus $95\%$ Aβ(1-42) $(5\%$ pE-Aβ) seed new cytotoxic LNOs
through multiple serial dilutions into Aβ(1-42) monomers in
the absence of additional Aβ(3(pE)-42). LNOs isolated from
human Alzheimer's disease brain contained Aβ(3(pE)-42), and
enhanced Aβ(3(pE)-42) formation in mice triggered neuron
loss and gliosis at 3 months, but not in a tau-null
background. We conclude that Aβ(3(pE)-42) confers
tau-dependent neuronal death and causes template-induced
misfolding of Aβ(1-42) into structurally distinct LNOs that
propagate by a prion-like mechanism. Our results raise the
possibility that Aβ(3(pE)-42) acts similarly at a primary
step in Alzheimer's disease pathogenesis.},
keywords = {Alzheimer Disease: metabolism / Amyloid: chemistry /
Amyloid: drug effects / Amyloid: metabolism / Amyloid:
toxicity / Amyloid beta-Peptides: chemistry / Amyloid
beta-Peptides: genetics / Amyloid beta-Peptides: metabolism
/ Amyloid beta-Peptides: toxicity / Animals / Disease
Models, Animal / Glutamic Acid: chemistry / Glutamic Acid:
metabolism / Humans / Mice / Mice, Transgenic / Mutant
Proteins: chemistry / Mutant Proteins: genetics / Mutant
Proteins: metabolism / Mutant Proteins: toxicity / Peptide
Fragments: chemistry / Peptide Fragments: genetics / Peptide
Fragments: metabolism / Peptide Fragments: toxicity /
Prions: chemistry / Prions: metabolism / Prions: toxicity /
tau Proteins: deficiency / tau Proteins: genetics / tau
Proteins: metabolism / Amyloid (NLM Chemicals) / Amyloid
beta-Peptides (NLM Chemicals) / Mutant Proteins (NLM
Chemicals) / Peptide Fragments (NLM Chemicals) / Prions (NLM
Chemicals) / amyloid beta-protein (1-42) (NLM Chemicals) /
tau Proteins (NLM Chemicals) / Glutamic Acid (NLM
Chemicals)},
cin = {AG Reymann},
ddc = {500},
cid = {I:(DE-2719)1310005},
pnm = {342 - Disease Mechanisms and Model Systems (POF3-342)},
pid = {G:(DE-HGF)POF3-342},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:22660329},
pmc = {pmc:PMC3367389},
doi = {10.1038/nature11060},
url = {https://pub.dzne.de/record/136529},
}