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@ARTICLE{Hartmann:282297,
author = {Hartmann, Christiane and Haß, Christina and Knobloch,
Muriel and Barrantes, Israel and Fumagalli, Laura and
Premereur, Jessie and Markert, Franz and Peters, Maite and
Koromila, Georgia and Hartmann, Alexander and Jäger,
Kathrin and Abel, Jette and Mancuso, Renzo and Storch,
Alexander and Walter, Michael and Fuellen, Georg and
Hermann, Andreas},
title = {{P}rematurely {A}ged {H}uman {M}icroglia {E}xhibit
{I}mpaired {S}tress {R}esponse and {D}efective
{N}ucleocytoplasmic {S}huttling of {ALS} {A}ssociated
{FUS}.},
journal = {Aging cell},
volume = {24},
number = {11},
issn = {1474-9718},
address = {Oxford [u.a.]},
publisher = {Wiley-Blackwell},
reportid = {DZNE-2025-01267},
pages = {e70232},
year = {2025},
abstract = {Microglia, the brain's resident immune cells, are crucial
for maintaining healthy brain homeostasis. However, as the
brain ages, microglia can shift from a neuroprotective to a
neurotoxic phenotype, contributing to chronic inflammation
and promoting neurodegenerative processes. Despite the
importance of understanding microglial aging, there are
currently few human in vitro models to study these
processes. To address this gap, we have developed a model in
which human microglia undergo accelerated aging through
inducible progerin expression. HMC3-Progerin cells display
key age-related markers such as activation of the
senescence-associated secretory phenotype (SASP) as well as
an increase in DNA damage. These prematurely aged HMC3 cells
show a reduced response to LPS activation, exhibit
impairments in essential microglial functions including
decreased migration and phagocytosis as well as
transcriptomic alterations including a shift observed in
aging and neurodegeneration. Additionally, we observed an
impaired stress response and a defect in nucleocytoplasmic
transport, especially affecting the amyotrophic lateral
sclerosis (ALS) associated protein FUS. This suggests that
microglia play a contributory role in driving
neurodegenerative processes in the aging brain. Our
microglia aging model offers a valuable tool for exploring
how aged microglia affect brain function, enhancing our
understanding of their role in brain aging.},
keywords = {Humans / Microglia: metabolism / Microglia: pathology /
Amyotrophic Lateral Sclerosis: metabolism / Amyotrophic
Lateral Sclerosis: pathology / Amyotrophic Lateral
Sclerosis: genetics / RNA-Binding Protein FUS: metabolism /
Cellular Senescence / Active Transport, Cell Nucleus /
Stress, Physiological / Phagocytosis / FUS (Other) / aging
(Other) / aging clock (Other) / amyotrophic lateral
sclerosis (Other) / microglia (Other) / nucleocytoplasmic
shuttling (Other) / progerin (Other) / senolytics (Other) /
RNA-Binding Protein FUS (NLM Chemicals)},
cin = {AG Hermann / AG Storch},
ddc = {610},
cid = {I:(DE-2719)1511100 / I:(DE-2719)5000014},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40970514},
pmc = {pmc:PMC12610945},
doi = {10.1111/acel.70232},
url = {https://pub.dzne.de/record/282297},
}