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@ARTICLE{Berghoff:155681,
author = {Berghoff, Stefan A and Spieth, Lena and Sun, Ting and
Hosang, Leon and Schlaphoff, Lennart and Depp, Constanze and
Düking, Tim and Winchenbach, Jan and Neuber, Jonathan and
Ewers, David and Scholz, Patricia and van der Meer,
Franziska and Cantuti-Castelvetri, Ludovico and Sasmita,
Andrew O and Meschkat, Martin and Ruhwedel, Torben and
Möbius, Wiebke and Sankowski, Roman and Prinz, Marco and
Huitinga, Inge and Sereda, Michael W and Odoardi, Francesca
and Ischebeck, Till and Simons, Mikael and
Stadelmann-Nessler, Christine and Edgar, Julia M and Nave,
Klaus-Armin and Saher, Gesine},
title = {{M}icroglia facilitate repair of demyelinated lesions via
post-squalene sterol synthesis.},
journal = {Nature neuroscience},
volume = {24},
number = {1},
issn = {1546-1726},
address = {New York, NY},
publisher = {Nature America},
reportid = {DZNE-2021-00849},
pages = {47 - 60},
year = {2021},
abstract = {The repair of inflamed, demyelinated lesions as in multiple
sclerosis (MS) necessitates the clearance of
cholesterol-rich myelin debris by microglia/macrophages and
the switch from a pro-inflammatory to an anti-inflammatory
lesion environment. Subsequently, oligodendrocytes increase
cholesterol levels as a prerequisite for synthesizing new
myelin membranes. We hypothesized that lesion resolution is
regulated by the fate of cholesterol from damaged myelin and
oligodendroglial sterol synthesis. By integrating gene
expression profiling, genetics and comprehensive
phenotyping, we found that, paradoxically, sterol synthesis
in myelin-phagocytosing microglia/macrophages determines the
repair of acutely demyelinated lesions. Rather than
producing cholesterol, microglia/macrophages synthesized
desmosterol, the immediate cholesterol precursor.
Desmosterol activated liver X receptor (LXR) signaling to
resolve inflammation, creating a permissive environment for
oligodendrocyte differentiation. Moreover, LXR target gene
products facilitated the efflux of lipid and cholesterol
from lipid-laden microglia/macrophages to support
remyelination by oligodendrocytes. Consequently,
pharmacological stimulation of sterol synthesis boosted the
repair of demyelinated lesions, suggesting novel therapeutic
strategies for myelin repair in MS.},
keywords = {Animals / Cholesterol: metabolism / Demyelinating Diseases:
pathology / Desmosterol: metabolism / Encephalomyelitis,
Autoimmune, Experimental / Female / Gene Expression
Profiling / Humans / Inflammation: metabolism /
Inflammation: pathology / Lipid Metabolism / Liver X
Receptors: metabolism / Mice / Mice, Inbred C57BL /
Microglia: physiology / Middle Aged / Multiple Sclerosis /
Oligodendroglia: metabolism / Phagocytosis / Squalene:
metabolism / Sterols: biosynthesis / Liver X Receptors (NLM
Chemicals) / Nr1h3 protein, mouse (NLM Chemicals) / Sterols
(NLM Chemicals) / Desmosterol (NLM Chemicals) / Squalene
(NLM Chemicals) / Cholesterol (NLM Chemicals)},
cin = {AG Simons},
ddc = {610},
cid = {I:(DE-2719)1110008},
pnm = {351 - Brain Function (POF4-351)},
pid = {G:(DE-HGF)POF4-351},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:33349711},
pmc = {pmc:PMC7116742},
doi = {10.1038/s41593-020-00757-6},
url = {https://pub.dzne.de/record/155681},
}