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@ARTICLE{Linsenmeier:473388,
author = {Linsenmeier, Luise and Mohammadi, Behnam and Shafiq, Mohsin
and Frontzek, Karl and Bär, Julia and Shrivastava, Amulya
N. and Damme, Markus and Song, Feizhi and Schwarz, Alexander
and Da Vela, Stefano and Massignan, Tania and Jung,
Sebastian and Correia, Angela and Schmitz, Matthias and
Puig, Berta and Hornemann, Simone and Zerr, Inga and
Tatzelt, Jörg and Biasini, Emiliano and Saftig, Paul and
Schweizer, Michaela and Svergun, Dmitri and Amin, Ladan and
Mazzola, Federica and Varani, Luca and Thapa, Simrika and
Gilch, Sabine and Schätzl, Hermann and Harris, David A. and
Triller, Antoine and Mikhaylova, Marina and Aguzzi, Adriano
and Altmeppen, Hermann C. and Glatzel, Markus},
title = {{L}igands binding to the prion protein induce its
proteolytic release with therapeutic potential in
neurodegenerative proteinopathies},
journal = {Science advances},
volume = {7},
number = {48},
issn = {2375-2548},
address = {Washington, DC [u.a.]},
publisher = {Assoc.},
reportid = {PUBDB-2022-00011},
pages = {eabj1826},
year = {2021},
abstract = {The prion protein (PrPC) is a central player in
neurodegenerative diseases, such as prion diseases or
Alzheimer’sdisease. In contrast to disease-promoting cell
surface PrPC, extracellular fragments act neuroprotective by
blockingneurotoxic disease-associated protein conformers.
Fittingly, PrPC release by the metalloprotease
ADAM10represents a protective mechanism. We used
biochemical, cell biological, morphological, and structural
methodsto investigate mechanisms stimulating this
proteolytic shedding. Shed PrP negatively correlates with
prion conversionand is markedly redistributed in murine
brain in the presence of prion deposits or amyloid plaques,
indicatinga sequestrating activity. PrP-directed ligands
cause structural changes in PrPC and increased shedding in
cellsand organotypic brain slice cultures. As an exception,
some PrP-directed antibodies targeting repetitive epitopesdo
not cause shedding but surface clustering, endocytosis, and
degradation of PrPC. Both mechanisms may contributeto
beneficial actions described for PrP-directed ligands and
pave the way for new therapeutic strategiesagainst currently
incurable neurodegenerative diseases.},
cin = {EMBL-User / EMBL},
ddc = {500},
cid = {I:(DE-H253)EMBL-User-20120814 / I:(DE-H253)EMBL-20120731},
pnm = {6G3 - PETRA III (DESY) (POF4-6G3) / DFG project 390688087 -
EXC 2049: Comprehensive approaches to neurological and
psychiatric disorders "NeuroCure" (390688087)},
pid = {G:(DE-HGF)POF4-6G3 / G:(GEPRIS)390688087},
experiment = {EXP:(DE-H253)P-P12-20150101},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:34818048},
UT = {WOS:000722215300013},
doi = {10.1126/sciadv.abj1826},
url = {https://bib-pubdb1.desy.de/record/473388},
}