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@ARTICLE{TayaranianMarvian:274025,
author = {Tayaranian Marvian, Amir and Strauß, Tabea and Tang, Qilin
and Tuck, Benjamin J and Keeling, Sophie and Rüdiger,
Daniel and Mirzazadeh Dizaji, Negar and Mohammad-Beigi,
Hossein and Nuscher, Brigitte and Chakraborty, Pijush and
Sutherland, Duncan S and McEwan, William A and Köglsperger,
Thomas and Zahler, Stefan and Zweckstetter, Markus and
Lichtenthaler, Stefan F and Wurst, Wolfgang and Schwarz,
Sigrid and Höglinger, Günter},
title = {{D}istinct regulation of {T}au {M}onomer and aggregate
uptake and intracellular accumulation in human neurons.},
journal = {Molecular neurodegeneration},
volume = {19},
number = {1},
issn = {1750-1326},
address = {London},
publisher = {Biomed Central},
reportid = {DZNE-2025-00006},
pages = {100},
year = {2024},
abstract = {The prion-like spreading of Tau pathology is the leading
cause of disease progression in various tauopathies. A
critical step in propagating pathologic Tau in the brain is
the transport from the extracellular environment and
accumulation inside naïve neurons. Current research
indicates that human neurons internalize both the
physiological extracellular Tau (eTau) monomers and the
pathological eTau aggregates. However, similarities or
differences in neuronal transport mechanisms between Tau
species remain elusive.Monomers, oligomers, and fibrils of
recombinant 2N4R Tau were produced and characterized by
biochemical and biophysical methods. A neuronal eTau uptake
and accumulation assay was developed for human induced
pluripotent stem cell-derived neurons (iPSCNs) and Lund
human mesencephalic cells (LUHMES)-derived neurons.
Mechanisms of uptake and cellular accumulation of eTau
species were studied by using small molecule inhibitors of
endocytic mechanisms and siRNAs targeting Tau uptake
mediators.Extracellular Tau aggregates accumulated more than
monomers in human neurons, mainly due to the higher
efficiency of small fibrillar and soluble oligomeric
aggregates in intraneuronal accumulation. A competition
assay revealed a distinction in the neuronal accumulation
between physiological eTau Monomers and pathology-relevant
aggregates, suggesting differential transport mechanisms.
Blocking heparan sulfate proteoglycans (HSPGs) with heparin
only inhibited the accumulation of eTau aggregates, whereas
monomers' uptake remained unaltered. At the molecular level,
the downregulation of genes involved in HSPG synthesis
exclusively blocked neuronal accumulation of eTau aggregates
but not monomers, suggesting its role in the transport of
pathologic Tau. Moreover, the knockdown of LRP1, as a
receptor of Tau, mainly reduced the accumulation of
monomeric form, confirming its involvement in Tau's
physiological transport.These data propose that despite the
similarity in the cellular mechanism, the uptake and
accumulation of eTau Monomers and aggregates in human
neurons are regulated by different molecular mediators.
Thus, they address the possibility of targeting the
pathological spreading of Tau aggregates without disturbing
the probable physiological or non-pathogenic transport of
Tau Monomers.},
keywords = {Humans / tau Proteins: metabolism / Neurons: metabolism /
Tauopathies: metabolism / Tauopathies: pathology / Induced
Pluripotent Stem Cells: metabolism / Protein Aggregates:
physiology / Cell-to-cell spreading (Other) / Extracellular
Tau (Other) / HSPGs (Other) / LRP1 (Other) /
Neurodegeneration (Other) / Uptake (Other) / VPS35 (Other) /
tau Proteins (NLM Chemicals) / Protein Aggregates (NLM
Chemicals) / MAPT protein, human (NLM Chemicals)},
cin = {Clinical Research (Munich) / AG Simons / AG Höglinger / AG
Zweckstetter / AG Lichtenthaler / AG Wurst},
ddc = {570},
cid = {I:(DE-2719)1111015 / I:(DE-2719)1110008 /
I:(DE-2719)1110002 / I:(DE-2719)1410001 / I:(DE-2719)1110006
/ I:(DE-2719)1140001},
pnm = {353 - Clinical and Health Care Research (POF4-353) / 351 -
Brain Function (POF4-351) / 352 - Disease Mechanisms
(POF4-352)},
pid = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-351 /
G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39736627},
pmc = {pmc:PMC11686972},
doi = {10.1186/s13024-024-00786-w},
url = {https://pub.dzne.de/record/274025},
}