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@ARTICLE{Khlwein:257780,
author = {Kühlwein, Julia and Ruf, Wolfgang and Kandler, Katharina
and Witzel, Simon and Lang, Christina and Mulaw, Medhanie A
and Ekici, Arif B and Weishaupt, Jochen H and Ludolph,
Albert C and Grozdanov, Veselin and Danzer, Karin M},
title = {{ALS} is imprinted in the chromatin accessibility of blood
cells.},
journal = {Cellular and molecular life sciences},
volume = {80},
number = {5},
issn = {1420-682X},
address = {Cham (ZG)},
publisher = {Springer International Publishing AG},
reportid = {DZNE-2023-00495},
pages = {131},
year = {2023},
abstract = {Amyotrophic Lateral Sclerosis (ALS) is a complex and
incurable neurodegenerative disorder in which genetic and
epigenetic factors contribute to the pathogenesis of all
forms of ALS. The interplay of genetic predisposition and
environmental footprints generates epigenetic signatures in
the cells of affected tissues, which then alter
transcriptional programs. Epigenetic modifications that
arise from genetic predisposition and systemic environmental
footprints should in theory be detectable not only in
affected CNS tissue but also in the periphery. Here, we
identify an ALS-associated epigenetic signature
('epiChromALS') by chromatin accessibility analysis of blood
cells of ALS patients. In contrast to the blood
transcriptome signature, epiChromALS includes also genes
that are not expressed in blood cells; it is enriched in CNS
neuronal pathways and it is present in the ALS motor cortex.
By combining simultaneous ATAC-seq and RNA-seq with
single-cell sequencing in PBMCs and motor cortex from ALS
patients, we demonstrate that epigenetic changes associated
with the neurodegenerative disease can be found in the
periphery, thus strongly suggesting a mechanistic link
between the epigenetic regulation and disease pathogenesis.},
keywords = {Humans / Amyotrophic Lateral Sclerosis: metabolism /
Epigenesis, Genetic / Chromatin / Genetic Predisposition to
Disease / Neurodegenerative Diseases: genetics / Blood
Cells: metabolism / Blood Cells: pathology / Chromatin
remodeling (Other) / Epigenome (Other) / Integrated analysis
(Other) / Motor neuron disease (Other) / Regulatory elements
(Other) / Single-nuclei sequencing (Other) / Chromatin (NLM
Chemicals)},
cin = {AG Danzer / Clinical Study Center Ulm},
ddc = {610},
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:37095391},
pmc = {pmc:PMC10126052},
doi = {10.1007/s00018-023-04769-w},
url = {https://pub.dzne.de/record/257780},
}