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@ARTICLE{Shen:142134,
author = {J. Shen$^*$ and S. Najafi$^*$ and S. Stäble$^*$ and J.
Fabian$^*$ and E. Koeneke$^*$ and F. Kolbinger$^*$ and J.
Wrobel$^*$ and B. Meder and M. Distel and T. Heimburg and W.
Sippl and M. Jung and H. Peterziel and D. Kranz$^*$ and M.
Boutros$^*$ and F. Westermann$^*$ and O. Witt$^*$ and I.
Oehme$^*$},
title = {{A} kinome-wide {RNA}i screen identifies {ALK} as a target
to sensitize neuroblastoma cells for {HDAC}8-inhibitor
treatment.},
journal = {Cell death and differentiation},
volume = {25},
number = {12},
issn = {1476-5403},
address = {London},
publisher = {Macmillan},
reportid = {DKFZ-2018-02364},
pages = {2053 - 2070},
year = {2018},
abstract = {The prognosis of advanced stage neuroblastoma patients
remains poor and, despite intensive therapy, the 5-year
survival rate remains less than $50\%.$ We previously
identified histone deacetylase (HDAC) 8 as an indicator of
poor clinical outcome and a selective drug target for
differentiation therapy in vitro and in vivo. Here, we
performed kinome-wide RNAi screening to identify genes that
are synthetically lethal with HDAC8 inhibitors. These
experiments identified the neuroblastoma predisposition gene
ALK as a candidate gene. Accordingly, the combination of the
ALK/MET inhibitor crizotinib and selective HDAC8 inhibitors
(3-6 µM PCI-34051 or 10 µM 20a) efficiently killed
neuroblastoma cell lines carrying wildtype ALK
(SK-N-BE(2)-C, IMR5/75), amplified ALK (NB-1), and those
carrying the activating ALK F1174L mutation (Kelly), and, in
cells carrying the activating R1275Q mutation (LAN-5),
combination treatment decreased viable cell count. The
effective dose of crizotinib in neuroblastoma cell lines
ranged from 0.05 µM (ALK-amplified) to 0.8 µM
(wildtype ALK). The combinatorial inhibition of ALK and
HDAC8 also decreased tumor growth in an in vivo zebrafish
xenograft model. Bioinformatic analyses revealed that the
mRNA expression level of HDAC8 was significantly correlated
with that of ALK in two independent patient cohorts, the
Academic Medical Center cohort (n = 88) and the German
Neuroblastoma Trial cohort (n = 649), and co-expression
of both target genes identified patients with very poor
outcome. Mechanistically, HDAC8 and ALK converge at the
level of receptor tyrosine kinase (RTK) signaling and their
downstream survival pathways, such as ERK signaling.
Combination treatment of HDAC8 inhibitor with crizotinib
efficiently blocked the activation of growth receptor
survival signaling and shifted the cell cycle arrest and
differentiation phenotype toward effective cell death of
neuroblastoma cell lines, including sensitization of
resistant models, but not of normal cells. These findings
reveal combined targeting of ALK and HDAC8 as a novel
strategy for the treatment of neuroblastoma.},
cin = {L101 / B110 / B087 / G340 / L601},
ddc = {610},
cid = {I:(DE-He78)L101-20160331 / I:(DE-He78)B110-20160331 /
I:(DE-He78)B087-20160331 / I:(DE-He78)G340-20160331 /
I:(DE-He78)L601-20160331},
pnm = {317 - Translational cancer research (POF3-317)},
pid = {G:(DE-HGF)POF3-317},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:29515255},
pmc = {pmc:PMC6261943},
doi = {10.1038/s41418-018-0080-0},
url = {https://inrepo02.dkfz.de/record/142134},
}