ArticleCancer research2015
Generation of a mouse model of atypical teratoid/rhabdoid tumor of the central nervous system through combined deletion of Snf5 and p53.
Article in Cancer research, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 1 of them a synthesis that pooled it.
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Who cites it
23 citing papers in PubMed, 1 synthesis or guideline pooled it, 45 citations in OpenAlex.
- Molecular subgrouping of atypical teratoid/rhabdoid tumors-a reinvestigation and current consensus.Neuro-oncology · 2020Pooled it
- Development of a Genetically Engineered Porcine Model of Rhabdoid Tumor Predisposition Syndrome Type 1 (RTPS-1).Cancers · 2026Article
- Targeting CDK11 in Rhabdoid Tumor of the Kidney.Cancers · 2026Article
- A cycling, progenitor-like cell population at the base of atypical teratoid rhabdoid tumor subtype differentiation trajectories.Neuro-oncology · 2025Article
- REST elevation-dependent chromatin remodeling and alternative Grk6 transcript synthesis hyperactivates Cxcr4-Sdf1 signaling in cerebellar granule cell progenitors.bioRxiv : the preprint server for biology · 2025Article
- NF-κB RelA regulates temporal oligodendrocyte differentiation in the postnatal brains.Frontiers in cellular neuroscience · 2025Article
- The evolutionary impact of childhood cancer on the human gene pool.Nature communications · 2024Article
- Modeling human brain rhabdoid tumor by inactivating tumor suppressor genes in induced pluripotent stem cells.Bioactive materials · 2024Article
- Modeling nervous system tumors with human stem cells and organoids.Cell regeneration (London, England) · 2023Review
- Developing Bottom-Up Induced Pluripotent Stem Cell Derived Solid Tumor Models Using Precision Genome Editing Technologies.The CRISPR journal · 2022Review
- Single-cell transcriptomics identifies potential cells of origin of MYC rhabdoid tumors.Nature communications · 2022Article
- The current landscape of immunotherapy for pediatric brain tumors.Nature cancer · 2022Review
- SWI/SNF chromatin remodeling complex is required for initiation of sex-dependent differentiation in mouse germline.Scientific reports · 2021Article
- Utilizing preclinical models to develop targeted therapies for rare central nervous system cancers.Neuro-oncology · 2021Review
- Polycomb repressive complex 2 in the driver's seat of childhood and young adult brain tumours.Trends in cell biology · 2021Review
- Delta-24-RGD, an Oncolytic Adenovirus, Increases Survival and Promotes Proinflammatory Immune Landscape Remodeling in Models of AT/RT and CNS-PNET.Clinical cancer research : an official journal of the American Association for Cancer Research · 2021Article
- Article
- Atypical Teratoid/Rhabdoid Tumor Originated From the Trigeminal Nerve in a Young Male Adult: Case Report and Review of the Literature.Frontiers in neurology · 2020Article
- Article
- Mutations in SMARCB1 and in other Coffin-Siris syndrome genes lead to various brain midline defects.Nature communications · 2019Article
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
Malignant rhabdoid tumors arise in several anatomic locations and are associated with poor outcomes. In the brain, these tumors are known as atypical teratoid/rhabdoid tumors (AT/RT). While genetically engineered models for malignant rhabdoid tumors exist, none of them recapitulate AT/RT, for which preclinical models remain lacking. In the majority of AT/RT, LOH occurs at the genetic locus SNF5 (Ini1/BAF47/Smarcb1), which functions as a subunit of the SWI/SNF chromatin-remodeling complex and a tumor suppressor in familial and sporadic malignant rhabdoid tumors. Therefore, we generated mice in which Snf5 was ablated specifically in nestin-positive and/or glial fibrillary acid protein (GFAP)-positive progenitor cells of the developing central nervous system (CNS). Snf5 ablation in nestin-positive cells resulted in early lethality that could not be rescued by loss of p53. However, Snf5 ablation in GFAP-positive cells caused a neurodegenerative phenotype exacerbated by p53 loss. Notably, these double mutants exhibited AT/RT development, associated with an earlier failure in granule neuron migration in the cerebellum, reduced neuronal projections in the hippocampus, degeneration of the corpus callosum, and ataxia and seizures. Gene expression analysis confirmed that the tumors that arose in Snf5/p53 mutant mice were distinct from other neural tumors and most closely resembled human AT/RT. Our findings uncover a novel role for Snf5 in oligodendrocyte generation and survival, and they offer evidence of the first genetically engineered mouse model for AT/RT in the CNS.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.