Highly penetrant, rapid tumorigenesis through conditional inversion of the tumor suppressor gene Snf5.

Roberts CW; Leroux MM; Fleming MD; Orkin SH

doi:10.1016/s1535-6108(02)00185-x

Highly penetrant, rapid tumorigenesis through conditional inversion of the tumor suppressor gene Snf5.

Affiliations

1. Division of Hematology-Oncology, Dana-Farber Cancer Institute and Children's Hospital, Department of Pediatrics, Harvard Medical School and Howard Hughes Medical Institute, Boston, MA 02115, USA.
Authors
Roberts CW¹
(1 author)

ORCIDs linked to this article

Cancer Cell, 01 Nov 2002, 2(5):415-425
https://doi.org/10.1016/s1535-6108(02)00185-x PMID: 12450796

Abstract

Recent data suggest the SWI/SNF chromatin remodeling complex may also act as a tumor suppressor. Utilizing a reversibly inactivating conditional allele, we demonstrate that loss of Snf5/Ini1/Baf47/SmarcB1, a core subunit of SWI/SNF, results in highly penetrant cancer predisposition with 100% of mice developing mature CD8(+) T cell lymphoma or rare rhabdoid tumors with a median onset of only 11 weeks. Notably, while loss of Snf5 predisposes to aggressive cancers, it is also required for survival of virtually all nonmalignant cells in vivo. Reversible gene targeting demonstrates a critical and specific role for Snf5 in tumor suppression, provides a novel system in which to explore the genetic pathways involved in tumor suppression by Swi/Snf, and should be of wide use in evaluating other essential tumor suppressor genes.

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Proteins in UniProt (Showing 5 of 14)

SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1(UniProt - F6U415)
SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1(UniProt - Q9ESU1)
SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1(UniProt - Q6ZWP4)
SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1(UniProt - Q9ESU0)
SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1(UniProt - D6RDC4)

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Mouse Genome Informatics (MGI)

http://www.informatics.jax.org/reference/12450796

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