ArticleCancer cell2019
p53 Is a Master Regulator of Proteostasis in SMARCB1-Deficient Malignant Rhabdoid Tumors.
Erratum issuedOpen access · greenAbstract read
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Article in Cancer cell, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 53 papers.
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53citing papers in PubMed
4.4field-weighted citation impact, top 5% of its field
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Who cites it
53 citing papers in PubMed, 88 citations in OpenAlex.
- CReP-mediated control of eIF2α phosphorylation is a critical node in MYC-driven medulloblastoma.Nature communications · 2026Article
- A Phase Ib Case Series Using Bortezomib With Chemotherapy in Atypical Teratoid/Rhabdoid Tumors.Cancer medicine · 2026Article
- Relapsed rhabdoid tumours and other non-nephroblastoma childhood and adolescent kidney tumours: perspectives from the HARMONICA collaboration.Nature reviews. Urology · 2026Review
- EphB4 inhibition defines a druggable synthetic-lethal vulnerability in MYC-driven triple-negative breast cancer.Cell death & disease · 2026Article
- SMARCA4 loss reprograms p300 chromatin occupancy to subvert p53-mediated transcriptional repression in ovarian small cell carcinoma.Nature communications · 2026Article
- Targeting CDK11 in Rhabdoid Tumor of the Kidney.Cancers · 2026Article
- Atypical teratoid rhabdoid tumor with extraneural metastases: a case report with genomic analysis.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Targeting the unfolded protein response in cancer: exploiting endoplasmic reticulum stress for therapeutic intervention.Biochemical pharmacology · 2025Review
- SMARCB1-related schwannomatosis and other SMARCB1-associated phenotypes: clinical spectrum and molecular pathogenesis.Familial cancer · 2025Review
- SIRT2 Regulates the SMARCB1 Loss-Driven Differentiation Block in ATRT.Molecular cancer research : MCR · 2025Article
- Super-enhancers and efficacy of triptolide in small cell carcinoma of the ovary hypercalcemic type.iScience · 2025Article
- SMARCB1-deficient poorly differentiated testicular carcinoma: a case report.Frontiers in oncology · 2025Article
- SMARCB1-deficient sinonasal adenocarcinoma: a rare variant of SWI/SNF-deficient malignancy often misclassified as high-grade non-intestinal-type sinonasal adenocarcinoma or myoepithelial carcinoma.Virchows Archiv : an international journal of pathology · 2024Article
- Targeting cholesterol biosynthesis for AT/RT: comprehensive expression analysis and validation in newly established AT/RT cell line.Human cell · 2024Article
- The IL6/JAK/STAT3 signaling axis is a therapeutic vulnerability in SMARCB1-deficient bladder cancer.Nature communications · 2024Article
- Genomic profiling and molecular characterization of non-clear cell renal cell carcinoma: a narrative review from a clinical perspective.Therapeutic advances in medical oncology · 2024Review
- A Carboxy-terminal Smarcb1 Point Mutation Induces Hydrocephalus Formation and Affects AP-1 and Neuronal Signalling Pathways in Mice.Cellular and molecular neurobiology · 2023Article
- Proteasome Inhibition Sensitizes Liposarcoma to MDM2 Inhibition with Nutlin-3 by Activating the ATF4/CHOP Stress Response Pathway.Cancer research · 2023Article
- (mis)-Targeting of SWI/SNF complex(es) in cancer.Cancer metastasis reviews · 2023Review
- SMARCB1 regulates a TFCP2L1-MYC transcriptional switch promoting renal medullary carcinoma transformation and ferroptosis resistance.Nature communications · 2023Article
Corrections and comments
- Erratum issued
Authors and funding
36 authors at 11 institutions in 4 countries.
Alessandro CarugoInstitute for Applied Cancer Science, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Rosalba MinelliDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Luigi SapioDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Melinda SoeungDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Federica CarboneDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Frederick S RobinsonInstitute for Applied Cancer Science, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
James TepperDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Ziheng ChenDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Sara LovisaDepartment of Cancer Biology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Maria SveltoTelethon Institute of Genetics and Medicine, Via Campi Flegrei 34, Pozzuoli 80078, Italy.
Samirkumar AminThe Jackson Laboratory for Genomic Medicine, 10 Discovery Drive, Farmington, CT 06032, USA.
Sanjana SrinivasanInstitute for Applied Cancer Science, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA; Department of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Edoardo Del PoggettoDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Sara LoponteDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Francesca PucaDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Prasenjit DeyDepartment of Cancer Biology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Gabriel G MaloufCentre Hospitalier Régional et Universitaire Strasbourg, Hôpital Civil, 1 Place de L'Hôpital, Strasbourg 67091, France; Department of Functional Genomics and Cancer, Institut de Génétique et de Biologie Moléculaire et Cellulaire, CNRS, INSERM, Université de Strasbourg, Illkirch 67400, France.
Xiaoping SuDepartment of Bioinformatics and Computational Biology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Liren LiSun Yat-sen University Cancer Center, State Key Laboratory of Oncology in South China, Guangzhou 510060, China.
Dolores Lopez-TerradaDepartment of Pathology, Texas Children's Hospital, 6621 Fannin Street, Houston, TX 77030, USA.
Dinesh RakhejaDepartment of Pathology, The University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA.
Alexander J LazarDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA; Department of Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
George J NettoDepartment of Pathology, Johns Hopkins University, 600 N. Wolfe Street/Carnegie 417, Baltimore, MD 21287, USA.
Priya RaoDepartment of Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Alessandro SgambatoDipartimento di Patologia Generale, Policlinico Agostino Gemelli, Università Cattolica del Sacro Cuore, Largo Francesco Vito 1, Roma 00168, Italy.
Anirban MaitraDepartment of Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA; Sheikh Ahmed Bin Zayed Al Nahyan Center for Pancreatic Cancer Research, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Durga N TripathiCenter for Precision Environmental Health, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
Cheryl L WalkerCenter for Precision Environmental Health, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
Jose A KaramDepartment of Urology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA; Department of Translational Molecular Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Timothy P HeffernanInstitute for Applied Cancer Science, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Andrea VialeDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Charles W M RobertsDepartment of Oncology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38120, USA.
Pavlos MsaouelDepartment of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Nizar M TannirDepartment of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA. Electronic address: ntannir@mdanderson.org.
Giulio F DraettaInstitute for Applied Cancer Science, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA; Department of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA. Electronic address: gdraetta@mdanderson.org.
Giannicola GenoveseDepartment of Genomic Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA; Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA; David H. Koch Center for Applied Research of Genitourinary Cancers, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA. Electronic address: ggenovese@mdanderson.org.
The University of Texas MD Anderson Cancer Center · USBaylor College of Medicine · USCentre National de la Recherche Scientifique · FRJackson Laboratory · USJohns Hopkins University · USSt. Jude Children's Research Hospital · USSun Yat-sen University · CNTelethon Institute Of Genetics And Medicine · ITTexas Children's Hospital · USThe University of Texas Southwestern Medical Center · USUniversità Cattolica del Sacro Cuore · IT
Funding
THE FUNCTION OF SNF5 (SMARCB1), AN EPIGENETIC TUMOR SUPPRESSORR01CA113794 · NCI · ST. JUDE CHILDREN'S RESEARCH HOSPITAL · PI CHARLES ROBERTS · 2005 to 2026
$7.8MTargeting Malic Enzyme 3 as a Synthetic Lethality Target in Pancreatic CancerR00CA218891 · NCI · ROSWELL PARK CANCER INSTITUTE CORP · PI DEY, PRASENJIT · 2019 to 2021
$747kTargeting malic enzyme 3 as a synthetic lethality target in pancreatic cancerK99CA218891 · NCI · UNIVERSITY OF TX MD ANDERSON CAN CTR · PI DEY, PRASENJIT · 2018 to 2019
$171kNCI NIH HHS K99 CA218891NCI NIH HHS R00 CA218891
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
Indexed as
AutophagyEndoplasmic Reticulum StressProteostasisAnimalsAntineoplastic AgentsCell Line, TumorCyclin-Dependent Kinase Inhibitor p16FemaleGene Expression Regulation, NeoplasticHumansMaleMice, 129 StrainMice, Inbred C57BLMice, KnockoutProteasome InhibitorsProto-Oncogene Proteins c-mycAntineoplastic AgentsCdkn2a protein, mouseCyclin-Dependent Kinase Inhibitor p16Myc protein, mouseProteasome InhibitorsProto-Oncogene Proteins c-mycSMARCB1 ProteinSMARCB1 protein, humanSmarcb1 protein, mouseTP53 protein, humanTrp53 protein, mouseTumor Suppressor Protein p53autophagyBIRC5embryonic mosaic GEM modelsER stressMYCp53proteasome inhibitorsrenal medullary carcinomarhabdoid tumorsSMARCB1
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