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@ARTICLE{Held:274095,
author = {Held, Sebastian and Erck, Christian and Kemppainen, Susanna
and Bleibaum, Florian and Giridhar, Neha Jadhav and
Feederle, Regina and Krenner, Claudia and Juopperi,
Sini-Pauliina and Calliari, Anna and Mentrup, Torben and
Schröder, Bernd and Dickson, Dennis W. and Rauramaa, Tuomas
and Petrucelli, Leonard and Prudencio, Mercedes and
Hiltunen, Mikko and Lüningschrör, Patrick and Capell, Anja
and Damme, Markus},
title = {{P}hysiological shedding and {C}-terminal proteolytic
processing of {TMEM}106{B}},
journal = {Cell reports},
volume = {44},
number = {1},
issn = {2211-1247},
address = {[New York, NY]},
publisher = {Elsevier},
reportid = {DZNE-2025-00051},
pages = {115107},
year = {2025},
abstract = {Genetic variants in TMEM106B, coding for a transmembrane
protein of unknown function, have been identified as
critical genetic modulators in various neurodegenerative
diseases with a strong effect in patients with
frontotemporal degeneration. The luminal domain of TMEM106B
can form amyloid-like fibrils upon proteolysis. Whether this
luminal domain is generated under physiological conditions
and which protease(s) are involved in shedding remain
unclear. We developed a commercially available antibody
against the luminal domain of TMEM106B, allowing a detailed
survey of the proteolytic processing under physiological
conditions in cellular models and TMEM106B-related mouse
models. Moreover, fibrillary TMEM106B was detected in human
autopsy material. We find that the luminal domain is
generated by multiple lysosomal cysteine-type proteases.
Cysteine-type proteases perform additional C-terminal
trimming, for which experimental evidence has been lacking.
The presented results allow an in-depth perception of the
processing of TMEM106B, a prerequisite to understanding
factors leading to fibril formation.},
cin = {AG Feederle / AG Haass},
ddc = {610},
cid = {I:(DE-2719)1140004 / I:(DE-2719)1110007},
pnm = {352 - Disease Mechanisms (POF4-352) / 899 - ohne Topic
(POF4-899)},
pid = {G:(DE-HGF)POF4-352 / G:(DE-HGF)POF4-899},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39709600},
doi = {10.1016/j.celrep.2024.115107},
url = {https://pub.dzne.de/record/274095},
}