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@ARTICLE{Scholz:130518,
author = {B. Scholz$^*$ and C. Korn$^*$ and J. Wojtarowicz$^*$ and C.
Mogler$^*$ and I. Augustin$^*$ and M. Boutros$^*$ and C.
Niehrs$^*$ and H. Augustin$^*$},
title = {{E}ndothelial {RSPO}3 {C}ontrols {V}ascular {S}tability and
{P}runing through {N}on-canonical {WNT}/{C}a(2+)/{NFAT}
{S}ignaling.},
journal = {Developmental cell},
volume = {36},
number = {1},
issn = {1534-5807},
address = {Cambridge, Mass.},
publisher = {Cell Press},
reportid = {DKFZ-2017-05597},
pages = {79 - 93},
year = {2016},
abstract = {The WNT signaling enhancer R-spondin3 (RSPO3)
is prominently expressed in the vasculature.
Correspondingly, embryonic lethality of Rspo3-deficient mice
is caused by vessel remodeling defects. Yet the mechanisms
underlying vascular RSPO3 function remain elusive. Inducible
endothelial Rspo3 deletion (Rspo3-iECKO) resulted in
perturbed developmental and tumor vascular remodeling.
Endothelial cell apoptosis and vascular pruning led to
reduced microvessel density in Rspo3-iECKO mice. Rspo3-iECKO
mice strikingly phenocopied the non-canonical WNT
signaling-induced vascular defects of mice deleted for the
WNT secretion factor Evi/Wls. An endothelial screen for
RSPO3 and EVI/WLS co-regulated genes identified Rnf213,
Usp18, and Trim30α. RNF213 targets filamin A and NFAT1 for
proteasomal degradation attenuating
non-canonical WNT/Ca(2+) signaling. Likewise, USP18 and
TRIM5α inhibited NFAT1 activation. Consequently, NFAT
protein levels were decreased in endothelial cells of
Rspo3-iECKO mice and pharmacological NFAT inhibition
phenocopied Rspo3-iECKO mice. The data identify endothelial
RSPO3-driven non-canonical WNT/Ca(2+)/NFAT signaling as a
critical maintenance pathway of the remodeling vasculature.},
keywords = {NFATC Transcription Factors (NLM Chemicals) / R-spondin3
protein, mouse (NLM Chemicals) / Thrombospondins (NLM
Chemicals) / Transcription Factors (NLM Chemicals) / Wnt
Proteins (NLM Chemicals) / Calcium (NLM Chemicals)},
cin = {A190 / B110 / A050 / L101},
ddc = {570},
cid = {I:(DE-He78)A190-20160331 / I:(DE-He78)B110-20160331 /
I:(DE-He78)A050-20160331 / I:(DE-He78)L101-20160331},
pnm = {321 - Basic Concepts (POF3-321)},
pid = {G:(DE-HGF)POF3-321},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:26766444},
doi = {10.1016/j.devcel.2015.12.015},
url = {https://inrepo02.dkfz.de/record/130518},
}