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@ARTICLE{Margreiter:906017,
author = {Margreiter, Michael A and Witzenberger, Monika and Wasser,
Yasmine and Davydova, Elena and Janowski, Robert and Metz,
Jonas and Habib, Pardes and Sahnoun, Sabri E. M. and
Sobisch, Carina and Poma, Benedetta and Palomino-Hernandez,
Oscar and Wagner, Mirko and Carell, Thomas and Shah, N. J.
and Schulz, Jörg B. and Niessing, Dierk and Voigt, Aaron
and Rossetti, Giulia},
title = {{S}mall-molecule modulators of {TRMT}2{A} decrease
{P}oly{Q} aggregation and {P}oly{Q}-induced cell death},
journal = {Computational and structural biotechnology journal},
volume = {20},
issn = {2001-0370},
address = {Gotenburg},
publisher = {Research Network of Computational and Structural
Biotechnology (RNCSB)},
reportid = {FZJ-2022-01182},
pages = {443 - 458},
year = {2022},
abstract = {Polyglutamine (polyQ) diseases are characterized by an
expansion of cytosine-adenine-guanine (CAG) trinucleotide
repeats encoding for an uninterrupted prolonged polyQ tract.
We previously identified TRMT2A as a strong modifier of
polyQ-induced toxicity in an unbiased large-scale screen in
Drosophila melanogaster. This work aimed at identifying and
validating pharmacological TRMT2A inhibitors as treatment
opportunities for polyQ diseases in humans. Computer-aided
drug discovery was implemented to identify human TRMT2A
inhibitors. Additionally, the crystal structure of one
protein domain, the RNA recognition motif (RRM), was
determined, and Biacore experiments with the RRM were
performed. The identified molecules were validated for their
potency to reduce polyQ aggregation and polyQ-induced cell
death in human HEK293T cells and patient derived
fibroblasts. Our work provides a first step towards
pharmacological inhibition of this enzyme and indicates
TRMT2A as a viable drug target for polyQ diseases.},
cin = {INM-4 / INM-11 / JARA-BRAIN / JSC / INM-9},
ddc = {570},
cid = {I:(DE-Juel1)INM-4-20090406 / I:(DE-Juel1)INM-11-20170113 /
I:(DE-Juel1)VDB1046 / I:(DE-Juel1)JSC-20090406 /
I:(DE-Juel1)INM-9-20140121},
pnm = {5253 - Neuroimaging (POF4-525) / 5111 - Domain-Specific
Simulation $\&$ Data Life Cycle Labs (SDLs) and Research
Groups (POF4-511)},
pid = {G:(DE-HGF)POF4-5253 / G:(DE-HGF)POF4-5111},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:35070167},
UT = {WOS:000819903300015},
doi = {10.1016/j.csbj.2021.12.029},
url = {https://juser.fz-juelich.de/record/906017},
}