% IMPORTANT: The following is UTF-8 encoded. This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.
@ARTICLE{Hautke:1019431,
author = {Hautke, Alexander and Voronin, Arthur and Idiris, Fathia
and Riel, Anton and Lindner, Felix and Lelièvre-Büttner,
Amandine and Zhu, Jikang and Appel, Bettina and Fatti,
Edoardo and Weis, Karsten and Müller, Sabine and Schug,
Alexander and Ebbinghaus, Simon},
title = {{CAG}-{R}epeat {RNA} {H}airpin {F}olding and {R}ecruitment
to {N}uclear {S}peckles with a {P}ivotal {R}ole of {ATP} as
a {C}osolute},
journal = {Journal of the American Chemical Society},
volume = {145},
number = {17},
issn = {0002-7863},
address = {Washington, DC},
publisher = {ACS Publications},
reportid = {FZJ-2023-05388},
pages = {9571 - 9583},
year = {2023},
abstract = {A hallmark of Huntington’s disease (HD) is a prolonged
polyglutamine sequence in the huntingtin protein and,
correspondingly, an expanded cytosine, adenine, and guanine
(CAG) triplet repeat region in the mRNA. A majority of
studies investigating disease pathology were concerned with
toxic huntingtin protein, but the mRNA moved into focus due
to its recruitment to RNA foci and emerging novel
therapeutic approaches targeting the mRNA. A hallmark of
CAG-RNA is that it forms a stable hairpin in vitro which
seems to be crucial for specific protein interactions. Using
in-cell folding experiments, we show that the CAG-RNA is
largely destabilized in cells compared to dilute buffer
solutions but remains folded in the cytoplasm and nucleus.
Surprisingly, we found the same folding stability in the
nucleoplasm and in nuclear speckles under physiological
conditions suggesting that CAG-RNA does not undergo a
conformational transition upon recruitment to the nuclear
speckles. We found that the metabolite adenosine
triphosphate (ATP) plays a crucial role in promoting
unfolding, enabling its recruitment to nuclear speckles and
preserving its mobility. Using in vitro experiments and
molecular dynamics simulations, we found that the ATP
effects can be attributed to a direct interaction of ATP
with the nucleobases of the CAG-RNA rather than ATP acting
as “a fuel” for helicase activity. ATP-driven changes in
CAG-RNA homeostasis could be disease-relevant since
mitochondrial function is affected in HD disease progression
leading to a decline in cellular ATP levels.},
cin = {JSC},
ddc = {540},
cid = {I:(DE-Juel1)JSC-20090406},
pnm = {5111 - Domain-Specific Simulation $\&$ Data Life Cycle Labs
(SDLs) and Research Groups (POF4-511) / Cells In Silico -
Large Scale Tissue Simulations $(cellsinsilico_20200501)$ /
GRK 2450 - GRK 2450: Maßgeschneiderte Multiskalenmethoden
für Computersimulationen von nanoskaligen Materialien
(389000774) / HAF - Helmholtz Analytics Framework
(ZT-I-0003)},
pid = {G:(DE-HGF)POF4-5111 / $G:(DE-Juel1)cellsinsilico_20200501$
/ G:(GEPRIS)389000774 / G:(DE-HGF)ZT-I-0003},
typ = {PUB:(DE-HGF)16},
pubmed = {37062072},
UT = {WOS:000974384000001},
doi = {10.1021/jacs.2c13653},
url = {https://juser.fz-juelich.de/record/1019431},
}