p53 Is a Master Regulator of Proteostasis in SMARCB1-Deficient Malignant Rhabdoid Tumors.
Document Type
Article
Publication Date
2-11-2019
Keywords
JGM
JAX Source
Cancer Cell 2019 Feb 11; 35(2):204-220.e9
Volume
35
Issue
2
First Page
204
Last Page
220
ISSN
1878-3686
PMID
30753823
DOI
https://doi.org/10.1016/j.ccell.2019.01.006
Abstract
Alterations in chromatin remodeling genes have been increasingly implicated in human oncogenesis. Specifically, the biallelic inactivation of the SWI/SNF subunit SMARCB1 results in the emergence of extremely aggressive pediatric malignancies. Here, we developed embryonic mosaic mouse models of malignant rhabdoid tumors (MRTs) that faithfully recapitulate the clinical-pathological features of the human disease. We demonstrated that SMARCB1-deficient malignancies exhibit dramatic activation of the unfolded protein response (UPR) and ER stress response via a genetically intact MYC-p19
Recommended Citation
Carugo A,
Minelli R,
Sapio L,
Soeung M,
Carbone F,
Robinson F,
Tepper J,
Chen Z,
Lovisa S,
Svelto M,
Amin S,
Srinivasan S,
Del Poggetto E,
Loponte S,
Puca F,
Dey P,
Malouf G,
Su X,
Li L,
Lopez-Terrada D,
Rakheja D,
Lazar A,
Netto G,
Rao P,
Sgambato A,
Maitra A,
Tripathi D,
Walker C,
Karam J,
Heffernan T,
Viale A,
Roberts C,
Msaouel P,
Tannir N,
Draetta G,
Genovese G.
p53 Is a Master Regulator of Proteostasis in SMARCB1-Deficient Malignant Rhabdoid Tumors. Cancer Cell 2019 Feb 11; 35(2):204-220.e9