% IMPORTANT: The following is UTF-8 encoded. This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.
@ARTICLE{Fabian:119916,
author = {J. Fabian$^*$ and M. Lodrini$^*$ and I. Oehme$^*$ and M. C.
Schier$^*$ and T. Thole$^*$ and T. Hielscher$^*$ and A.
Kopp-Schneider$^*$ and L. Opitz and D. Capper$^*$ and A. von
Deimling$^*$ and I. Wiegand$^*$ and T. Milde$^*$ and U.
Mahlknecht and F. Westermann$^*$ and O. Popanda$^*$ and F.
Roels and B. Hero and F. Berthold and M. Fischer and A. E.
Kulozik and O. Witt$^*$ and H. E. Deubzer$^*$},
title = {{GRHL}1 acts as tumor suppressor in neuroblastoma and is
negatively regulated by {MYCN} and {HDAC}3.},
journal = {Cancer research},
volume = {74},
number = {9},
issn = {1538-7445},
address = {Philadelphia, Pa.},
publisher = {AACR},
reportid = {DKFZ-2017-00507},
pages = {2604 - 2616},
year = {2014},
abstract = {Neuroblastoma is an embryonic solid tumor of neural crest
origin and accounts for $11\%$ of all cancer-related deaths
in children. Novel therapeutic strategies are therefore
urgently required. MYCN oncogene amplification, which occurs
in $20\%$ of neuroblastomas, is a hallmark of high risk.
Here, we aimed to exploit molecular mechanisms that can be
pharmacologically addressed with epigenetically modifying
drugs, such as histone deacetylase (HDAC) inhibitors.
Grainyhead-like 1 (GRHL1), a gene critical for Drosophila
neural development, belonged to the genes most strongly
responding to HDAC inhibitor treatment of neuroblastoma
cells in a genome-wide screen. An increase in the histone H4
pan-acetylation associated with its promoter preceded
transcriptional activation. Physically adjacent, HDAC3 and
MYCN colocalized to the GRHL1 promoter and repressed its
transcription. High-level GRHL1 expression in primary
neuroblastomas correlated on transcriptional and
translational levels with favorable patient survival and
established clinical and molecular markers for favorable
tumor biology, including lack of MYCN amplification.
Enforced GRHL1 expression in MYCN-amplified neuroblastoma
cells with low endogenous GRHL1 levels abrogated
anchorage-independent colony formation, inhibited
proliferation, and retarded xenograft growth in mice. GRHL1
knockdown in MYCN single-copy cells with high endogenous
GRHL1 levels promoted colony formation. GRHL1 regulated 170
genes genome-wide, and most were involved in pathways
regulated during neuroblastomagenesis, including nervous
system development, proliferation, cell-cell adhesion, cell
spreading, and cellular differentiation. In summary, the
data presented here indicate a significant role of HDAC3 in
the MYCN-mediated repression of GRHL1 and suggest drugs that
block HDAC3 activity and suppress MYCN expression as
promising candidates for novel treatment strategies of
high-risk neuroblastoma.},
keywords = {Antineoplastic Agents (NLM Chemicals) / GRHL1 protein,
human (NLM Chemicals) / Histone Deacetylase Inhibitors (NLM
Chemicals) / Hydroxamic Acids (NLM Chemicals) / Indoles (NLM
Chemicals) / MYCN protein, human (NLM Chemicals) / N-Myc
Proto-Oncogene Protein (NLM Chemicals) / Nuclear Proteins
(NLM Chemicals) / Oncogene Proteins (NLM Chemicals) /
Repressor Proteins (NLM Chemicals) / panobinostat (NLM
Chemicals) / Histone Deacetylases (NLM Chemicals) / histone
deacetylase 3 (NLM Chemicals)},
cin = {G340 / C060 / G380 / B087 / C010},
ddc = {610},
cid = {I:(DE-He78)G340-20160331 / I:(DE-He78)C060-20160331 /
I:(DE-He78)G380-20160331 / I:(DE-He78)B087-20160331 /
I:(DE-He78)C010-20160331},
pnm = {317 - Translational cancer research (POF3-317)},
pid = {G:(DE-HGF)POF3-317},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:24419085},
doi = {10.1158/0008-5472.CAN-13-1904},
url = {https://inrepo02.dkfz.de/record/119916},
}