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@ARTICLE{Jethwa:136739,
author = {A. Jethwa$^*$ and M. Słabicki$^*$ and J. Hüllein$^*$ and
M. Jentzsch$^*$ and V. Dalal$^*$ and S. Rabe and L.
Wagner$^*$ and T. Walther$^*$ and W. Klapper and H.
Bohnenberger and M. Rettel and J. Lu and A. H. Smits and F.
Stein and M. M. Savitski and W. Huber and Y. Aylon and M.
Oren and T. Zenz$^*$},
collaboration = {M. N. Project},
title = {{TRRAP} is essential for regulating the accumulation of
mutant and wild-type p53 in lymphoma.},
journal = {Blood},
volume = {131},
number = {25},
issn = {1528-0020},
address = {Stanford, Calif.},
publisher = {HighWire Press},
reportid = {DKFZ-2018-01177},
pages = {2789 - 2802},
year = {2018},
abstract = {Tumors accumulate high levels of mutant p53 (mutp53), which
contributes to mutp53 gain-of-function properties. The
mechanisms that underlie such excessive accumulation are not
fully understood. To discover regulators of mutp53 protein
accumulation, we performed a large-scale RNA interference
screen in a Burkitt lymphoma cell line model. We identified
transformation/transcription domain-associated protein
(TRRAP), a constituent of several histone acetyltransferase
complexes, as a critical positive regulator of both mutp53
and wild-type p53 levels. TRRAP silencing attenuated p53
accumulation in lymphoma and colon cancer models, whereas
TRRAP overexpression increased mutp53 levels, suggesting a
role for TRRAP across cancer entities and p53 mutations.
Through clustered regularly interspaced short palindromic
repeats (CRISPR)-Cas9 screening, we identified a
109-amino-acid region in the N-terminal HEAT repeat region
of TRRAP that was crucial for mutp53 stabilization and cell
proliferation. Mass spectrometric analysis of the mutp53
interactome indicated that TRRAP silencing caused
degradation of mutp53 via the MDM2-proteasome axis. This
suggests that TRRAP is vital for maintaining mutp53 levels
by shielding it against the natural p53 degradation
machinery. To identify drugs that alleviated p53
accumulation similarly to TRRAP silencing, we performed a
small-molecule drug screen and found that inhibition of
histone deacetylases (HDACs), specifically HDAC1/2/3,
decreased p53 levels to a comparable extent. In summary,
here we identify TRRAP as a key regulator of p53 levels and
link acetylation-modifying complexes to p53 protein
stability. Our findings may provide clues for therapeutic
targeting of mutp53 in lymphoma and other cancers.},
cin = {G250 / G100},
ddc = {610},
cid = {I:(DE-He78)G250-20160331 / I:(DE-He78)G100-20160331},
pnm = {317 - Translational cancer research (POF3-317)},
pid = {G:(DE-HGF)POF3-317},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:29653964},
doi = {10.1182/blood-2017-09-806679},
url = {https://inrepo02.dkfz.de/record/136739},
}