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@ARTICLE{Eberhard:276798,
author = {Eberhard, Judith and Henning, Lukas and Fülle, Lorenz and
Knöpper, Konrad and Böhringer, Jana and Graelmann,
Frederike and Hänschke, Lea and Kenzler, Julia and
Brosseron, Frederic and Heneka, Michael and Domingos, Ana I
and Eyerich, Stefanie and Lochner, Matthias and Weighardt,
Heike and Bedner, Peter and Steinhäuser, Christian and
Förster, Irmgard},
title = {{A}blation of {CCL}17-positive hippocampal neurons induces
inflammation-dependent epilepsy.},
journal = {Epilepsia},
volume = {66},
number = {2},
issn = {0013-9580},
address = {Oxford [u.a.]},
publisher = {Wiley-Blackwell},
reportid = {DZNE-2025-00323},
pages = {554 - 568},
year = {2025},
abstract = {Neuronal cell death and neuroinflammation are
characteristic features of epilepsy, but it remains unclear
whether neuronal cell death as such is causative for the
development of epileptic seizures. To test this hypothesis,
we established a novel mouse line permitting inducible
ablation of pyramidal neurons by inserting simian diphtheria
toxin (DT) receptor (DTR) cDNA into the Ccl17 locus. The
chemokine CCL17 is expressed in pyramidal CA1 neurons in
adult mice controlling microglial quiescence.Seizure
activity in CCL17-DTR mice was analyzed by
electroencephalographic recordings following treatment with
DT for 3 consecutive days. Neuroinflammation and neuronal
cell death were evaluated by (immuno)histochemistry.
Pharmacological inhibition of TNFR1 signaling was achieved
by treatment with XPro1595, a dominant-negative inhibitor of
soluble tumor necrosis factor.Neuronal cell death was
detectable 7 days (d7) after the first DT injection in
heterozygous CCL17-DTR mice. Spontaneous epileptic seizures
were observed in the vast majority of mice, often with an
initial peak at d6-9, followed by a period of reduced
activity and a gradual increase during the 1-month
observation period. Microglial reactivity was overt from d5
after DT administration not only in the CA1 region but also
in the CA2/CA3 area, shortly followed by astrogliosis.
Reactive microgliosis and astrogliosis persisted until d30
and, together with neuronal loss and stratum radiatum
shrinkage, reflected important features of human hippocampal
sclerosis. Granule cell dispersion was detectable only 3
months after DT treatment. Application of XPro1595
significantly reduced chronic seizure burden without
affecting the development of hippocampal sclerosis.In
conclusion, our data demonstrate that sterile pyramidal
neuronal death is sufficient to cause epilepsy in the
absence of other pathological processes. The CCL17-DTR mouse
line may thus be a valuable model for further mechanistic
studies on epilepsy and assessment of antiseizure
medication.},
keywords = {Animals / Mice / Epilepsy: pathology / Epilepsy: drug
therapy / Hippocampus: pathology / Hippocampus: drug effects
/ Hippocampus: metabolism / Neurons: drug effects / Neurons:
pathology / Neurons: metabolism / Mice, Transgenic /
Chemokine CCL17: genetics / Chemokine CCL17: metabolism /
Electroencephalography / Cell Death: drug effects / Mice,
Inbred C57BL / Male / Disease Models, Animal / Microglia:
drug effects / Microglia: metabolism / Microglia: pathology
/ Pyramidal Cells: drug effects / Pyramidal Cells:
metabolism / Pyramidal Cells: pathology / Inflammation:
pathology / Inflammation: metabolism / XPro1595 (Other) /
astrogliosis (Other) / diphtheria toxin (Other) /
microgliosis (Other) / neurodegeneration (Other) / Chemokine
CCL17 (NLM Chemicals) / Ccl17 protein, mouse (NLM
Chemicals)},
cin = {AG Heneka},
ddc = {610},
cid = {I:(DE-2719)1011303},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39607395},
pmc = {pmc:PMC11827734},
doi = {10.1111/epi.18200},
url = {https://pub.dzne.de/record/276798},
}