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A genetic analysis of pax3-fkhr, the oncogene of alveolar rhabdomyosarcoma

Academic Article
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Overview

authors

  • Kempf, B. E.
  • Vogt, Peter K.

publication date

  • December 1999

journal

  • Cell Growth & Differentiation  Journal

abstract

  • The PAX3-FKHR fusion protein of human alveolar rhabdomyosarcoma consists of the DNA-binding domains of PAX3 and the transcriptional activation domain of FKHR. It induces oncogenic transformation in cultures of chicken embryo fibroblasts (CEFs). PAX3-FKHR-transformed CEFs have been kept in continuous culture for more than 1 year; when quiescent, portions of the cultures differentiate into several distinct cell types. Deletion analysis suggests that both DNA binding and transcriptional activation are required for the induction of the PAX3-FKHR-transformed cellular phenotype. Mutant PAX3-FKHR proteins with reduced DNA binding or transactivation induce altered cellular morphologies and growth behavior distinct from that of CEFs expressing wild-type PAX3-FKHR. Mutant proteins that completely lack DNA binding or transactivation potential fail to transform.

subject areas

  • Animals
  • Cell Transformation, Neoplastic
  • Chick Embryo
  • DNA
  • DNA-Binding Proteins
  • Fibroblasts
  • Forkhead Transcription Factors
  • Humans
  • Mutagenesis
  • Oncogene Proteins
  • Paired Box Transcription Factors
  • Recombinant Fusion Proteins
  • Rhabdomyosarcoma, Alveolar
  • Sequence Deletion
  • Transcription Factors
  • Transcriptional Activation
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Identity

International Standard Serial Number (ISSN)

  • 1044-9523

PubMed ID

  • 10616906
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Additional Document Info

start page

  • 813

end page

  • 818

volume

  • 10

issue

  • 12

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