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@ARTICLE{Rahman:257584,
author = {Rahman, Kazi Atikur and Orlando, Marta and Boulos, Ayub and
Andrzejak, Ewa and Schmitz, Dietmar and Ziv, Noam E and
Prüss, Harald and Garner, Craig C and Ichkova, Aleksandra},
title = {{M}icroglia actively remove {NR}1 autoantibody-bound {NMDA}
receptors and associated post-synaptic proteins in neuron
microglia co-cultures.},
journal = {Glia},
volume = {71},
number = {8},
issn = {0894-1491},
address = {Bognor Regis [u.a.]},
publisher = {Wiley-Liss},
reportid = {DZNE-2023-00460},
pages = {1804-1829},
year = {2023},
abstract = {Autoantibodies against the NR1 subunit of NMDA receptors
(NMDARs) have been shown to promote crosslinking and
internalization of bound receptors in NMDAR encephalitis
(NMDARE). This internalization-mediated loss of NMDARs is
thought to be the major mechanism leading to pathogenic
outcomes in patients. However, the role of bound
autoantibody in engaging the resident immune cells,
microglia, remains poorly understood. Here, using a
patient-derived monoclonal NR1 autoantibody (hNR1-mAb) and a
co-culture system of microglia and neurons, we could show
that hNR1-mAb bound to hippocampal neurons led to
microglia-mediated removal of hNR1-mAb bound NMDARs. These
complexes were found to accumulate inside endo-lysosomal
compartments of microglia. Utilizing another patient
isolated monoclonal autoantibody, against the α1-subunit of
GABAA receptors (α1-GABAA -mAb), such removal of receptors
was found to be specific to the antibody-bound receptor
targets. Interestingly, along with receptor removal, we also
observed a reduction in synapse number, more specifically in
the numbers of post-synaptic proteins like PSD95 and Homer
1, when microglia were present in the culture. Importantly,
mutations in the Fc region of hNR1-mAb, blocking its Fcγ
receptor (FcγR) and complement binding, attenuated hNR1-mAb
driven loss of NMDARs and synapses, indicating that
microglia engagement by bound hNR1-mAb is critical for
receptor and synapse loss. Our data argues for an active
involvement of microglia in removal of NMDARs and other
receptors in individuals with autoimmune encephalitis,
thereby contributing to the etiology of these diseases.},
keywords = {Humans / Receptors, N-Methyl-D-Aspartate: genetics /
Receptors, N-Methyl-D-Aspartate: metabolism /
Autoantibodies: metabolism / Coculture Techniques /
Microglia: metabolism / Neurons: metabolism /
gamma-Aminobutyric Acid: metabolism / co-culture (Other) /
NMDAR (Other) / antibody mediated autoimmune encephalitis
(Other) / autoantibodies (Other) / microglia, hippocampal
neurons, co-culture, pre-labeling (Other) / hippocampal
neurons (Other) / microglia (Other) / pre-labeling (Other) /
Receptors, N-Methyl-D-Aspartate (NLM Chemicals) /
Autoantibodies (NLM Chemicals) / gamma-Aminobutyric Acid
(NLM Chemicals)},
cin = {AG Garner / AG Schmitz 1 ; AG Schmitz / AG Prüß},
ddc = {610},
cid = {I:(DE-2719)1810001 / I:(DE-2719)1810004 /
I:(DE-2719)1810003},
pnm = {351 - Brain Function (POF4-351) / 353 - Clinical and Health
Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-351 / G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37026600},
doi = {10.1002/glia.24369},
url = {https://pub.dzne.de/record/257584},
}