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@ARTICLE{Cukkemane:1043477,
author = {Cukkemane, Abhishek and Becker, Nina and Kupreichyk,
Tatsiana and Heise, Henrike and Willbold, Dieter and
Weiergräber, Oliver H.},
title = {{T}racing the aggregation pathway of the scaffold protein
{DISC}1: {S}tructural implications for chronic mental
illnesses},
journal = {Journal of structural biology: X},
volume = {11},
issn = {2590-1524},
address = {Amsterdam},
publisher = {Elsevier},
reportid = {FZJ-2025-02880},
pages = {100128 -},
year = {2025},
abstract = {Disrupted in schizophrenia 1 (DISC1) is a pleiotropic
scaffold protein that is postulated to comprise large
disordered regions and four distinct structured segments
with a high proportion of helical or coiled-coil fold. DISC1
associates with over 300 proteins and is associated with
several physiological roles ranging from mitosis to cellular
differentiation. Yet, the structural features of the protein
are poorly characterized. The C-terminal region (C-region,
res. 691–836) forms a tetramer and can also aggregate into
amyloid-like fibers, potentially linked to schizophrenia and
other chronic mental illnesses. Using a combination of
biophysical and structural biology applications, we
investigate the structural heterogeneity of three mutants of
the C-region, viz., the S713E, S704C and L807-frameshift
mutants. We provide evidence for the plasticity of the C
region; a thin border separates the conformational
flexibility of DISC1 required for interaction with a myriad
of partners from disruptive aggregation. Snapshots of
aggregates and fibrils growing from a nucleus are presented,
along with data supporting the role of the minimal
fibrillizing element in the C-region, the β-core. This
segment also houses a stretch of residues that is critical
for the binding of NDEL1 proteins in the mitotic spindle
complex and is absent in the non-binding splice variant
DISC1Δ22aa. Physiologically, both the splice variant and
the fibers represent loss-of-function states that disrupt
cellular division. Our findings highlight the need to
decipher the structural elements within the DISC1 C-region
to comprehend its physiological role and aggregation-related
anomalies, and to establish a rationale for drug
development.},
cin = {IBI-7},
ddc = {624},
cid = {I:(DE-Juel1)IBI-7-20200312},
pnm = {5244 - Information Processing in Neuronal Networks
(POF4-524)},
pid = {G:(DE-HGF)POF4-5244},
typ = {PUB:(DE-HGF)16},
pubmed = {40503182},
UT = {WOS:001501725600001},
doi = {10.1016/j.yjsbx.2025.100128},
url = {https://juser.fz-juelich.de/record/1043477},
}