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@ARTICLE{Snuderl:136814,
author = {M. Snuderl and K. Kannan and E. Pfaff$^*$ and S. Wang and
J. M. Stafford and J. Serrano and A. Heguy and K. Ray and A.
Faustin and O. Aminova and I. Dolgalev and S. L. Stapleton
and D. Zagzag and L. Chiriboga and S. L. Gardner and J. H.
Wisoff and J. G. Golfinos and D. Capper$^*$ and V.
Hovestadt$^*$ and M. K. Rosenblum and D. G. Placantonakis
and S. E. LeBoeuf and T. Y. Papagiannakopoulos and L.
Chavez$^*$ and S. Ahsan and C. G. Eberhart and S.
Pfister$^*$ and D. Jones$^*$ and M. A. Karajannis},
title = {{R}ecurrent homozygous deletion of {DROSHA} and
microduplication of {PDE}4{DIP} in pineoblastoma.},
journal = {Nature Communications},
volume = {9},
number = {1},
issn = {2041-1723},
address = {London},
publisher = {Nature Publishing Group},
reportid = {DKFZ-2018-01252},
pages = {2868},
year = {2018},
abstract = {Pineoblastoma is a rare and highly aggressive brain cancer
of childhood, histologically belonging to the spectrum of
primitive neuroectodermal tumors. Patients with germline
mutations in DICER1, a ribonuclease involved in microRNA
processing, have increased risk of pineoblastoma, but
genetic drivers of sporadic pineoblastoma remain unknown.
Here, we analyzed pediatric and adult pineoblastoma samples
(n = 23) using a combination of genome-wide DNA
methylation profiling and whole-exome sequencing or
whole-genome sequencing. Pediatric and adult pineoblastomas
showed distinct methylation profiles, the latter clustering
with lower-grade pineal tumors and normal pineal gland.
Recurrent variants were found in genes involved in PKA- and
NF-κB signaling, as well as in chromatin remodeling genes.
We identified recurrent homozygous deletions of DROSHA,
acting upstream of DICER1 in microRNA processing, and a
novel microduplication involving chromosomal region 1q21
containing PDE4DIP (myomegalin), comprising the ancient
DUF1220 protein domain. Expresion of PDE4DIP and DUF1220
proteins was present exclusively in pineoblastoma with
PDE4DIP gain.},
cin = {B062 / G380 / L101 / B060},
ddc = {500},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)G380-20160331 /
I:(DE-He78)L101-20160331 / I:(DE-He78)B060-20160331},
pnm = {319H - Addenda (POF3-319H)},
pid = {G:(DE-HGF)POF3-319H},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:30030436},
pmc = {pmc:PMC6054684},
doi = {10.1038/s41467-018-05029-3},
url = {https://inrepo02.dkfz.de/record/136814},
}