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@ARTICLE{Briel:165111,
author = {Briel, Nils and Ruf, Viktoria C and Pratsch, Katrin and
Roeber, Sigrun and Widmann, Jeannine and Mielke, Janina and
Dorostkar, Mario Manucehr and Windl, Otto and Arzberger,
Thomas and Herms, Jochen and Strübing, Felix},
title = {{S}ingle-nucleus chromatin accessibility profiling
highlights distinct astrocyte signatures in progressive
supranuclear palsy and corticobasal degeneration.},
journal = {Acta neuropathologica},
volume = {144},
number = {4},
issn = {0001-6322},
address = {Heidelberg},
publisher = {Springer},
reportid = {DZNE-2022-01420},
pages = {615 - 635},
year = {2022},
abstract = {Tauopathies such as progressive supranuclear palsy (PSP)
and corticobasal degeneration (CBD) exhibit characteristic
neuronal and glial inclusions of hyperphosphorylated Tau
(pTau). Although the astrocytic pTau phenotype upon
neuropathological examination is the most guiding feature in
distinguishing both diseases, regulatory mechanisms
controlling their transitions into disease-specific states
are poorly understood to date. Here, we provide accessible
chromatin data of more than 45,000 single nuclei isolated
from the frontal cortex of PSP, CBD, and control
individuals. We found a strong association of
disease-relevant molecular changes with astrocytes and
demonstrate that tauopathy-relevant genetic risk variants
are tightly linked to astrocytic chromatin accessibility
profiles in the brains of PSP and CBD patients. Unlike the
established pathogenesis in the secondary tauopathy
Alzheimer disease, microglial alterations were relatively
sparse. Transcription factor (TF) motif enrichments in
pseudotime as well as modeling of the astrocytic TF
interplay suggested a common pTau signature for CBD and PSP
that is reminiscent of an inflammatory immediate-early
response. Nonetheless, machine learning models also
predicted discriminatory features, and we observed marked
differences in molecular entities related to protein
homeostasis between both diseases. Predicted TF involvement
was supported by immunofluorescence analyses in postmortem
brain tissue for their highly correlated target genes.
Collectively, our data expand the current knowledge on risk
gene involvement (e.g., MAPT, MAPK8, and NFE2L2) and
molecular pathways leading to the phenotypic changes
associated with CBD and PSP.},
keywords = {Astrocytes: pathology / Chromatin / Corticobasal
Degeneration / Humans / Supranuclear Palsy, Progressive:
pathology / Tauopathies: genetics / Tauopathies: pathology /
tau Proteins: genetics / tau Proteins: metabolism /
Astrocytes (Other) / Corticobasal degeneration (Other) /
Neurodegeneration (Other) / Progressive supranuclear palsy
(Other) / Tauopathy (Other) / snATAC-seq (Other)},
cin = {AG Herms / Neuropathology / Brainbank},
ddc = {610},
cid = {I:(DE-2719)1110001 / I:(DE-2719)1140013},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pmc = {pmc:PMC9468099},
pubmed = {pmid:35976433},
doi = {10.1007/s00401-022-02483-8},
url = {https://pub.dzne.de/record/165111},
}