p53 Is a Master Regulator of Proteostasis in SMARCB1-Deficient Malignant Rhabdoid Tumors.

Animals Antineoplastic Agents / pharmacology Autophagy / drug effects Cell Line, Tumor Cyclin-Dependent Kinase Inhibitor p16 / genetics Endoplasmic Reticulum Stress / drug effects Female Gene Expression Regulation, Neoplastic Humans Male Mice, 129 Strain Mice, Inbred C57BL Mice, Knockout Proteasome Inhibitors / pharmacology Proteostasis / drug effects Proto-Oncogene Proteins c-myc / genetics Rhabdoid Tumor / drug therapy SMARCB1 Protein / deficiency Signal Transduction Tumor Cells, Cultured Tumor Suppressor Protein p53 / deficiency Unfolded Protein Response

BIRC5 ER stress MYC SMARCB1 autophagy embryonic mosaic GEM models p53 proteasome inhibitors renal medullary carcinoma rhabdoid tumors

Journal

Cancer cell

ISSN: 1878-3686

Titre abrégé: Cancer Cell

Pays: United States

ID NLM: 101130617

Informations de publication

Date de publication:
11 02 2019

Historique:

received: 28 03 2018

revised: 12 10 2018

accepted: 09 01 2019

entrez: 13 2 2019

pubmed: 13 2 2019

medline: 18 12 2019

Statut: ppublish

Résumé

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

Identifiants

DOI: 10.1016/j.ccell.2019.01.006 PMID: 30753823 PMC: PMC7876656

pubmed: 30753823

pii: S1535-6108(19)30040-6

doi: 10.1016/j.ccell.2019.01.006

pmc: PMC7876656

mid: NIHMS1664392

pii:

doi:

Substances chimiques

Antineoplastic Agents 0

Cdkn2a protein, mouse 0

Cyclin-Dependent Kinase Inhibitor p16 0

Myc protein, mouse 0

Proteasome Inhibitors 0

Proto-Oncogene Proteins c-myc 0

SMARCB1 Protein 0

SMARCB1 protein, human 0

Smarcb1 protein, mouse 0

TP53 protein, human 0

Trp53 protein, mouse 0

Tumor Suppressor Protein p53 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S.

Langues

eng

Sous-ensembles de citation

Pagination

204-220.e9

Subventions

Organisme : NCI NIH HHS

ID : K99 CA218891

Pays : United States

Organisme : NCI NIH HHS

ID : R00 CA218891

Pays : United States

Informations de copyright

Références

J Clin Invest. 2012 Aug;122(8):2983-8

pubmed: 22797305

Nat Neurosci. 2010 Jan;13(1):133-40

pubmed: 20023653

Cell. 2016 Apr 21;165(3):631-42

pubmed: 27062928

Mol Biol Cell. 2008 Mar;19(3):797-806

pubmed: 18094039

Cell. 2014 Oct 9;159(2):440-55

pubmed: 25263330

Cell. 2006 Jun 30;125(7):1253-67

pubmed: 16814713

J Clin Oncol. 2005 Mar 1;23(7):1491-9

pubmed: 15735125

Cancer Cell. 2002 Nov;2(5):415-25

pubmed: 12450796

Nature. 2013 Jul 11;499(7457):214-218

pubmed: 23770567

Nature. 2012 Mar 28;483(7391):603-7

pubmed: 22460905

Proc Natl Acad Sci U S A. 2009 May 12;106(19):7852-7

pubmed: 19416861

Cancer. 2012 Sep 1;118(17):4212-9

pubmed: 22213196

Pediatr Surg Int. 2009 Nov;25(11):923-9

pubmed: 19701757

N Engl J Med. 2003 Jun 26;348(26):2609-17

pubmed: 12826635

Nature. 2017 Feb 16;542(7641):362-366

pubmed: 28178232

Nature. 2011 May 5;473(7345):101-4

pubmed: 21471965

Pediatr Blood Cancer. 2017 Nov;64(11):

pubmed: 28485059

CA Cancer J Clin. 2004 Jul-Aug;54(4):208-36

pubmed: 15253918

Bioinformatics. 2004 Feb 12;20(3):307-15

pubmed: 14960456

EMBO J. 2005 Sep 21;24(18):3313-24

pubmed: 16138077

Cell Rep. 2016 Apr 26;15(4):801-813

pubmed: 27149838

Hepatology. 2006 Dec;44(6):1478-86

pubmed: 17133486

Blood. 2014 Aug 14;124(7):1047-55

pubmed: 24904120

Nucleic Acids Res. 2015 Apr 20;43(7):e47

pubmed: 25605792

Cancer Cell. 2016 Mar 14;29(3):394-406

pubmed: 26977886

Cell Rep. 2012 Aug 30;2(2):386-96

pubmed: 22884371

Clin Cancer Res. 2011 Jan 1;17(1):31-8

pubmed: 21208904

Nat Biotechnol. 2000 May;18(5):538-43

pubmed: 10802622

Cancer Res. 2008 Nov 15;68(22):9459-68

pubmed: 19010921

Proc Natl Acad Sci U S A. 2000 Dec 5;97(25):13796-800

pubmed: 11095756

Nature. 1995 Oct 19;377(6550):646-9

pubmed: 7566179

Nature. 1998 Jul 9;394(6689):203-6

pubmed: 9671307

Cell. 2008 Aug 8;134(3):451-60

pubmed: 18692468

Nat Genet. 2016 Jan;48(1):4-6

pubmed: 26711108

Gene Ther. 2000 Dec;7(23):1986-92

pubmed: 11175309

Nat Commun. 2016 Jan 28;7:10421

pubmed: 26818002

Nat Genet. 2003 Jul;34(3):267-73

pubmed: 12808457

Proc Natl Acad Sci U S A. 2005 Oct 25;102(43):15545-50

pubmed: 16199517

Pediatr Blood Cancer. 2012 Dec 15;59(7):1155-7

pubmed: 22997201

Pediatr Blood Cancer. 2009 Mar;52(3):328-34

pubmed: 18985717

Nucleic Acids Res. 2013 Jan;41(Database issue):D955-61

pubmed: 23180760

Genes Dev. 2000 Apr 15;14(8):994-1004

pubmed: 10783170

Cell. 2004 Dec 17;119(6):847-60

pubmed: 15607980

J Neuropathol Exp Neurol. 2011 Jul;70(7):596-609

pubmed: 21666498

Ann Rheum Dis. 2010 Jan;69(1):20-8

pubmed: 19103632

Proc Natl Acad Sci U S A. 2005 Dec 6;102(49):17745-50

pubmed: 16301525

Genes Dev. 2001 Dec 15;15(24):3243-8

pubmed: 11751630

Oncol Rep. 2008 Mar;19(3):819-23

pubmed: 18288421

EMBO Rep. 2000 Dec;1(6):500-6

pubmed: 11263494

Eur Urol. 2016 Jun;69(6):1055-61

pubmed: 26433572

Cell. 2006 Jul 14;126(1):121-34

pubmed: 16839881

Nat Methods. 2011 Dec 04;9(1):81-3

pubmed: 22138822

Nature. 2015 Dec 3;528(7580):84-7

pubmed: 26570998

Cancer Cell. 2016 Mar 14;29(3):379-393

pubmed: 26923874

Dis Model Mech. 2012 May;5(3):397-402

pubmed: 22228755

Eur J Cancer. 2016 Jun;60:69-82

pubmed: 27082136

Pediatr Blood Cancer. 2011 Sep;57(3):423-8

pubmed: 21744471

Genes Dev. 2002 Nov 15;16(22):2958-70

pubmed: 12435636

CA Cancer J Clin. 2018 Mar;68(2):133-152

pubmed: 29377070

Cancer Cell. 2016 Dec 12;30(6):891-908

pubmed: 27960086