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@ARTICLE{Lauinger:282502,
author = {M. Lauinger and D. Christen$^*$ and R. Klar$^*$ and C.
Roubaty and C. Heilig$^*$ and M. Stumpe and J. J. Knox and
N. Radulovich and L. Tamblyn and I. Y. Xie and P. Horak$^*$
and A. Forschner$^*$ and M. Bitzer$^*$ and U. A. Wittel and
M. Börries$^*$ and C. Ball$^*$ and C. Heining$^*$ and H.
Glimm$^*$ and M. Fröhlich and D. Hübschmann$^*$ and S.
Gallinger and R. Fritsch and S. Fröhling$^*$ and G. M.
O'Kane and J. Dengjel and T. Brummer$^*$},
title = {{BRAF}Δβ3-α{C} in-frame deletion mutants differ in their
dimerization propensity, {HSP}90 dependence, and
druggability.},
journal = {Science advances},
volume = {9},
number = {35},
issn = {2375-2548},
address = {Washington, DC [u.a.]},
publisher = {Assoc.},
reportid = {DKFZ-2023-01791},
pages = {eade7486},
year = {2023},
abstract = {In-frame BRAF exon 12 deletions are increasingly identified
in various tumor types. The resultant BRAFΔβ3-αC
oncoproteins usually lack five amino acids in the β3-αC
helix linker and sometimes contain de novo insertions. The
dimerization status of BRAFΔβ3-αC oncoproteins, their
precise pathomechanism, and their direct druggability by RAF
inhibitors (RAFi) has been under debate. Here, we
functionally characterize BRAFΔLNVTAP>F and two novel
mutants, BRAFdelinsFS and BRAFΔLNVT>F, and compare them
with other BRAFΔβ3-αC oncoproteins. We show that
BRAFΔβ3-αC oncoproteins not only form stable homodimers
and large multiprotein complexes but also require
dimerization. Nevertheless, details matter as aromatic amino
acids at the deletion junction of some BRAFΔβ3-αC
oncoproteins, e.g., BRAFΔLNVTAP>F, increase their stability
and dimerization propensity while conferring resistance to
monomer-favoring RAFi such as dabrafenib or HSP 90/CDC37
inhibition. In contrast, dimer-favoring inhibitors such as
naporafenib inhibit all BRAFΔβ3-αC mutants in cell lines
and patient-derived organoids, suggesting that tumors driven
by such oncoproteins are vulnerable to these compounds.},
cin = {FR01 / B340 / HD01 / TU01 / DD01},
ddc = {500},
cid = {I:(DE-He78)FR01-20160331 / I:(DE-He78)B340-20160331 /
I:(DE-He78)HD01-20160331 / I:(DE-He78)TU01-20160331 /
I:(DE-He78)DD01-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37656784},
doi = {10.1126/sciadv.ade7486},
url = {https://inrepo02.dkfz.de/record/282502},
}