The Hybrid PAX3-FKHR Fusion Protein of Alveolar Rhabdomyosarcoma Transforms Fibroblasts in Culture

Pediatric alveolar rhabdomyosarcoma is characterized by a chromosomal translocation that fuses parts of the PAX3 and FKHR genes. PAX3 codes for a transcriptional regulator that controls developmental programs, and FKHR codes for a forkhead-winged helix protein, also a likely transcription factor. Th...

Full description

Saved in:
Bibliographic Details
Published in:Proceedings of the National Academy of Sciences - PNAS 1996-09, Vol.93 (18), p.9805-9809
Main Authors: Scheidler, Sabine, Fredericks, William J., Rauscher, Frank J., Barr, Frederic G., Vogt, Peter K.
Format: Article
Language:English
Subjects:
Animals
Blotting, Northern
Cell Transformation, Neoplastic
Cells, Cultured
Chick Embryo
DNA
DNA-Binding Proteins - pharmacology
Embryos
Fibroblasts
Forkhead Box Protein O1
Forkhead Transcription Factors
Genes
Humans
Oncogenes
Paired Box Transcription Factors
PAX3 Transcription Factor
Poultry
Proteins
Regulator genes
Rhabdomyosarcoma
Rhabdomyosarcoma, Alveolar - genetics
RNA
Transcription factors
Transcription Factors - pharmacology
Transcriptional regulatory elements
Transfection
Translocation, Genetic
Tumors
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Pediatric alveolar rhabdomyosarcoma is characterized by a chromosomal translocation that fuses parts of the PAX3 and FKHR genes. PAX3 codes for a transcriptional regulator that controls developmental programs, and FKHR codes for a forkhead-winged helix protein, also a likely transcription factor. The PAX3-FKHR fusion product retains the DNA binding domains of the PAX3 protein and the putative activator domain of the FKHR protein. The PAX3-FKHR protein has been shown to function as a transcriptional activator. Using the RCAS retroviral vector, we have introduced the PAX3-FKHR gene into chicken embryo fibroblasts. Expression of the PAX3-FKHR protein in these cells leads to transformation: the cells become enlarged, grow tightly packed and in multiple layers, and acquire the ability for anchorage-independent growth. This cellular transformation in vitro will facilitate studies on the mechanism of PAX3-FKHR-induced oncogenesis.
ISSN:0027-8424
1091-6490
DOI:10.1073/pnas.93.18.9805