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@ARTICLE{Ruf:285638,
author = {Ruf, Wolfgang P and Kühlwein, Julia and Meier, Laura and
Tripke, Sarah and LeeBae, Jaehyun and Sadri-Vakili, Ghazaleh
and Yilmazer-Hanke, Deniz and Petri, Susanne and Thal,
Dietmar R and Grozdanov, Veselin and Danzer, Karin M},
title = {{M}ulti-modal dissection of cell-type specific {TDP}-43
pathology in the motor cortex.},
journal = {Nature Communications},
volume = {17},
number = {1},
issn = {2041-1723},
address = {[London]},
publisher = {Springer Nature},
reportid = {DZNE-2026-00277},
pages = {2406},
year = {2026},
abstract = {Cytoplasmic TDP-43 pathology is a pathological sign of
ALS/ALS-FTD and a converging disease event across different
genotypes, phenotypes and CNS areas. To understand this
process and target it therapeutically, we need to define
which cell types are affected and which cell-type specific
effects make them particularly vulnerable. We coupled
flow-cytometry nuclear sorting and sequencing with
single-nucleus multi-omic ATAC-seq and RNA-seq and spatial
transcriptomics to define the transcriptional cell type of
affected neurons in the post-mortem ALS/ALS-FTD motor cortex
(30 ALS, 20 ALS-FTD $\&$ 32 control samples). Here, we show
that mainly excitatory cortical neurons are affected by
TDP-43 pathology and define the cell types that are affected
the most: intratelencephalic L2-L3-LINC00507-FREM3,
L3-L5-RORB-LNX2, L3-L5-RORB-ADGRL4 $\&$ L6-THEMIS-LINC00343
neurons and extratelencephalic L5-FEZF2-NTNG1 neurons.
Transcriptional aberrations by TDP-43 pathology, like
cryptic exon inclusion, are cell-type specific and affect
distinct gene sets in each cell type, highlighting the need
to address TDP-43 pathology in a cell-type specific manner.},
keywords = {Motor Cortex: pathology / Motor Cortex: metabolism / Humans
/ DNA-Binding Proteins: metabolism / DNA-Binding Proteins:
genetics / Amyotrophic Lateral Sclerosis: pathology /
Amyotrophic Lateral Sclerosis: genetics / Amyotrophic
Lateral Sclerosis: metabolism / Male / Neurons: metabolism /
Neurons: pathology / Female / Frontotemporal Dementia:
pathology / Frontotemporal Dementia: genetics /
Frontotemporal Dementia: metabolism / Aged / Middle Aged /
Transcriptome / DNA-Binding Proteins (NLM Chemicals) /
TARDBP protein, human (NLM Chemicals)},
cin = {AG Danzer / Clinical Study Center (Ulm)},
ddc = {500},
cid = {I:(DE-2719)5000072 / I:(DE-2719)5000077},
pnm = {352 - Disease Mechanisms (POF4-352) / 353 - Clinical and
Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-352 / G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:41803120},
doi = {10.1038/s41467-026-69944-6},
url = {https://pub.dzne.de/record/285638},
}