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Related Experiment Video

Updated: Jun 27, 2026

Identification and Characterization of Metastatic Factors by Gene Transfer into the Novel RIP-Tag; RIP-tva Murine Model
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Identification and Characterization of Metastatic Factors by Gene Transfer into the Novel RIP-Tag; RIP-tva Murine Model

Published on: October 16, 2017

Development of a Genetically Engineered Porcine Model of Rhabdoid Tumor Predisposition Syndrome Type 1 (RTPS-1).

Brian Na1,2,3, C Dustin Rubinstein4, Jennifer J Meudt5

  • 1Department of Head and Neck Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA 90095, USA.

Cancers
|June 26, 2026
PubMed
Summary

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Researchers developed the first porcine model for Atypical Teratoid Rhabdoid Tumors (ATRT) by targeting the SMARCB1 gene. This new large animal model shows promise for studying ATRT and testing new treatments.

Area of Science:

  • Oncology
  • Genetics
  • Animal Models

Background:

  • Atypical Teratoid Rhabdoid Tumors (ATRT) are aggressive CNS malignancies primarily affecting infants, often linked to SMARCB1 gene disruption.
  • Existing mouse models inadequately replicate human ATRT's clinical and biological aspects, limiting therapeutic outcome prediction.
  • The rarity of ATRT complicates clinical trial patient cohort assembly, hindering treatment progress.

Purpose of the Study:

  • To establish a large animal model for SMARCB1-deficient rhabdoid tumors using pigs, given the high conservation of the porcine SMARCB1 gene.
  • To investigate the potential of germline deletion of SMARCB1 exons 4 and 5 in predisposing swine to ATRT development.

Main Methods:

  • Utilized CRISPR/Cas9 gene editing to create a porcine model with targeted deletion of SMARCB1 exons 4 and 5.
Keywords:
CRISPR/Cas9pig modelrhabdoid tumors

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Last Updated: Jun 27, 2026

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Published on: October 16, 2017

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  • Crossed a pig with SMARCB1 exon deletion with one carrying a TP53 exon 2 truncation to enhance tumorigenicity.
  • Monitored piglets for tumor development and genetic alterations.

    • Main Results:

    • Two piglets exhibited confirmed SMARCB1 targeted excisions, but none spontaneously developed tumors.
    • A subsequent cross resulted in piglets with SMARCB1 exon deletion; one developed a spinal mass, indicating tumor formation.
    • This study reports the first porcine model for ATRT, demonstrating proof-of-concept for large animal modeling of these tumors.

    Conclusions:

    • The developed porcine model represents a significant advancement for studying SMARCB1-deficient rhabdoid tumors.
    • This model offers a promising platform for preclinical research and therapeutic strategy evaluation for ATRT.
    • Further development of this porcine model (RTPS-1) is supported for advancing ATRT preclinical applications.