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@ARTICLE{Woo:280787,
author = {Woo, Marcel S and Brand, Johannes and Bal, Lukas C and
Moritz, Manuela and Walkenhorst, Mark and Vieira, Vanessa
and Ipenberg, Inbal and Rothammer, Nicola and Wang, Man and
Dogan, Batuhan and Loreth, Desirée and Mayer, Christina and
Nagel, Darwin and Wagner, Ingrid and Pfeffer, Lena Kristina
and Landgraf, Peter and van Ham, Marco and Mattern, Kuno M-J
and Winschel, Ingo and Frantz, Noah and Sonner, Jana K and
Grosshans, Henrike K and Miguela, Albert and Bauer, Simone
and Meurs, Nina and Müller, Anke and Binkle-Ladisch, Lars
and Salinas, Gabriela and Jänsch, Lothar and Dieterich,
Daniela C and Riedner, Maria and Krüger, Elke and Heppner,
Frank L and Glatzel, Markus and Puelles, Victor G and
Engler, Jan Broder and Nyengaard, Jens Randel and Misgeld,
Thomas and Kerschensteiner, Martin and Merkler, Doron and
Meyer-Schwesinger, Catherine and Friese, Manuel A},
title = {{T}he immunoproteasome disturbs neuronal metabolism and
drives neurodegeneration in multiple sclerosis.},
journal = {Cell},
volume = {188},
number = {17},
issn = {0092-8674},
address = {[Cambridge, Mass.]},
publisher = {Cell Press},
reportid = {DZNE-2025-00971},
pages = {4567 - 4585.e32},
year = {2025},
abstract = {Inflammation, aberrant proteostasis, and energy depletion
are hallmarks of neurodegenerative diseases such as multiple
sclerosis (MS). However, the interplay between inflammation,
proteasomal dysfunction in neurons, and its consequences for
neuronal integrity remains unclear. Using transcriptional,
proteomic, and functional analyses of proteasomal subunits
in inflamed neurons, we found that interferon-γ-mediated
induction of the immunoproteasome subunit, proteasome 20S
beta 8 (PSMB8) impairs the proteasomal balance, resulting in
reduced proteasome activity. This reduction causes the
accumulation of
phosphofructo-2-kinase/fructose-2,6-bisphosphatase 3
(PFKFB3), a key metabolic regulator, leading to enhanced
neuronal glycolysis, reduced pentose phosphate pathway
activity, oxidative injury, and ferroptosis. Neuron-specific
genetic and systemic pharmacological targeting of PSMB8 or
PFKFB3 protected neurons in vitro and in a mouse model of
MS. Our findings provide a unifying explanation for
proteasomal dysfunction in MS and possibly other
neurodegenerative diseases, linking inflammation to
metabolic disruption, and presenting an opportunity for
targeted neuroprotective therapies.},
keywords = {Animals / Proteasome Endopeptidase Complex: metabolism /
Proteasome Endopeptidase Complex: genetics / Multiple
Sclerosis: metabolism / Multiple Sclerosis: pathology /
Multiple Sclerosis: immunology / Neurons: metabolism /
Neurons: pathology / Mice / Phosphofructokinase-2:
metabolism / Glycolysis / Humans / Interferon-gamma:
metabolism / Mice, Inbred C57BL / Inflammation: metabolism /
Disease Models, Animal / Pentose Phosphate Pathway /
Ferroptosis / Neurodegenerative Diseases: metabolism / Male
/ Female / Encephalomyelitis, Autoimmune, Experimental /
excitotoxicity (Other) / ferroptosis (Other) / glycolysis
(Other) / immunoproteasome (Other) / interferon-γ (Other) /
metabolism (Other) / multiple sclerosis (Other) /
neurodegeneration (Other) / neuroinflammation (Other) /
Proteasome Endopeptidase Complex (NLM Chemicals) /
Phosphofructokinase-2 (NLM Chemicals) / Interferon-gamma
(NLM Chemicals) / PFKFB3 protein, mouse (NLM Chemicals) /
Psmb10 protein, mouse (NLM Chemicals)},
cin = {AG Heppner / AG Misgeld},
ddc = {610},
cid = {I:(DE-2719)1810007 / I:(DE-2719)1110000-4},
pnm = {353 - Clinical and Health Care Research (POF4-353) / 351 -
Brain Function (POF4-351)},
pid = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-351},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40532699},
doi = {10.1016/j.cell.2025.05.029},
url = {https://pub.dzne.de/record/280787},
}