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@ARTICLE{CaldiGomes:270146,
author = {Caldi Gomes, Lucas and Hänzelmann, Sonja and Hausmann,
Fabian and Khatri, Robin and Oller, Sergio and Parvaz, Mojan
and Tzeplaeff, Laura and Pasetto, Laura and Gebelin, Marie
and Ebbing, Melanie and Holzapfel, Constantin and Columbro,
Stefano Fabrizio and Scozzari, Serena and Knöferle, Johanna
and Cordts, Isabell and Demleitner, Antonia F and Deschauer,
Marcus and Dufke, Claudia and Sturm, Marc and Zhou, Qihui
and Zelina, Pavol and Sudria-Lopez, Emma and Haack, Tobias B
and Streb, Sebastian and Kuzma-Kozakiewicz, Magdalena and
Edbauer, Dieter and Pasterkamp, R Jeroen and Laczko, Endre
and Rehrauer, Hubert and Schlapbach, Ralph and Carapito,
Christine and Bonetto, Valentina and Bonn, Stefan and
Lingor, Paul},
title = {{M}ultiomic {ALS} signatures highlight subclusters and sex
differences suggesting the {MAPK} pathway as therapeutic
target.},
journal = {Nature Communications},
volume = {15},
number = {1},
issn = {2041-1723},
address = {[London]},
publisher = {Nature Publishing Group UK},
reportid = {DZNE-2024-00744},
pages = {4893},
year = {2024},
abstract = {Amyotrophic lateral sclerosis (ALS) is a debilitating motor
neuron disease and lacks effective disease-modifying
treatments. This study utilizes a comprehensive multiomic
approach to investigate the early and sex-specific molecular
mechanisms underlying ALS. By analyzing the prefrontal
cortex of 51 patients with sporadic ALS and 50 control
subjects, alongside four transgenic mouse models (C9orf72-,
SOD1-, TDP-43-, and FUS-ALS), we have uncovered significant
molecular alterations associated with the disease. Here, we
show that males exhibit more pronounced changes in molecular
pathways compared to females. Our integrated analysis of
transcriptomes, (phospho)proteomes, and miRNAomes also
identified distinct ALS subclusters in humans, characterized
by variations in immune response, extracellular matrix
composition, mitochondrial function, and RNA processing. The
molecular signatures of human subclusters were reflected in
specific mouse models. Our study highlighted the
mitogen-activated protein kinase (MAPK) pathway as an early
disease mechanism. We further demonstrate that trametinib, a
MAPK inhibitor, has potential therapeutic benefits in vitro
and in vivo, particularly in females, suggesting a direction
for developing targeted ALS treatments.},
keywords = {Amyotrophic Lateral Sclerosis: genetics / Amyotrophic
Lateral Sclerosis: drug therapy / Amyotrophic Lateral
Sclerosis: metabolism / Humans / Female / Animals / Male /
Mice / Mice, Transgenic / MAP Kinase Signaling System: drug
effects / Disease Models, Animal / Pyridones: pharmacology /
Pyridones: therapeutic use / RNA-Binding Protein FUS:
metabolism / RNA-Binding Protein FUS: genetics / Prefrontal
Cortex: metabolism / Transcriptome / Superoxide Dismutase-1:
genetics / Superoxide Dismutase-1: metabolism / DNA-Binding
Proteins: metabolism / DNA-Binding Proteins: genetics /
Middle Aged / MicroRNAs: genetics / MicroRNAs: metabolism /
C9orf72 Protein: genetics / C9orf72 Protein: metabolism /
Sex Characteristics / Aged / Sex Factors / Pyrimidinones /
trametinib (NLM Chemicals) / Pyridones (NLM Chemicals) /
RNA-Binding Protein FUS (NLM Chemicals) / Superoxide
Dismutase-1 (NLM Chemicals) / DNA-Binding Proteins (NLM
Chemicals) / MicroRNAs (NLM Chemicals) / C9orf72 Protein
(NLM Chemicals) / TARDBP protein, human (NLM Chemicals) /
SOD1 protein, human (NLM Chemicals) / FUS protein, human
(NLM Chemicals) / Pyrimidinones (NLM Chemicals)},
cin = {Clinical Research (Munich) / AG Zhou / AG Edbauer},
ddc = {500},
cid = {I:(DE-2719)1111015 / I:(DE-2719)5000080 /
I:(DE-2719)1110004},
pnm = {353 - Clinical and Health Care Research (POF4-353) / 352 -
Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:38849340},
pmc = {pmc:PMC11161513},
doi = {10.1038/s41467-024-49196-y},
url = {https://pub.dzne.de/record/270146},
}