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@ARTICLE{Zoltsman:289082,
author = {G. Zoltsman and T. L. Dang$^*$ and M. Kuchersky and O.
Faust and M. S. Silva and T. Ilani and A. S. Wentink and B.
Bukau$^*$ and R. Rosenzweig},
title = {{A} unique chaperoning mechanism in class {A} {JDP}s
recognizes and stabilizes mutant p53.},
journal = {Molecular cell},
volume = {84},
number = {8},
issn = {1097-2765},
address = {New York, NY},
publisher = {Elsevier},
reportid = {DKFZ-2024-00582},
pages = {1512-1526.e9},
year = {2024},
note = {DKFZ-ZMBH-Alliance / 2024 Apr 18;84(8):1512-1526.e9},
abstract = {J-domain proteins (JDPs) constitute a large family of
molecular chaperones that bind a broad spectrum of
substrates, targeting them to Hsp70, thus determining the
specificity of and activating the entire chaperone
functional cycle. The malfunction of JDPs is therefore
inextricably linked to myriad human disorders. Here, we
uncover a unique mechanism by which chaperones recognize
misfolded clients, present in human class A JDPs. Through a
newly identified β-hairpin site, these chaperones detect
changes in protein dynamics at the initial stages of
misfolding, prior to exposure of hydrophobic regions or
large structural rearrangements. The JDPs then sequester
misfolding-prone proteins into large oligomeric assemblies,
protecting them from aggregation. Through this mechanism,
class A JDPs bind destabilized p53 mutants, preventing
clearance of these oncoproteins by Hsp70-mediated
degradation, thus promoting cancer progression. Removal of
the β-hairpin abrogates this protective activity while
minimally affecting other chaperoning functions. This
suggests the class A JDP β-hairpin as a highly specific
target for cancer therapeutics.},
keywords = {J-domain proteins (Other) / NMR (Other) / molecular
chaperones (Other) / p53 (Other) / protein aggregation
(Other) / protein misfolding (Other)},
cin = {A250},
ddc = {610},
cid = {I:(DE-He78)A250-20160331},
pnm = {311 - Zellbiologie und Tumorbiologie (POF4-311)},
pid = {G:(DE-HGF)POF4-311},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:38508184},
doi = {10.1016/j.molcel.2024.02.018},
url = {https://inrepo02.dkfz.de/record/289082},
}