Pediatric cancer gene database (Pedican) Home
Pedican
Pediatric cancer database
General information | Literature | Expression | Regulation | Mutation | Interaction

Basic Information

Gene ID

1958

Name

EGR1

Synonymous

AT225|G0S30|KROX-24|NGFI-A|TIS8|ZIF-268|ZNF225;early growth response 1;EGR1;early growth response 1

Definition

EGR-1|early growth response protein 1|nerve growth factor-induced protein A|transcription factor ETR103|transcription factor Zif268|zinc finger protein 225|zinc finger protein Krox-24

Position

5q31.1

Gene type

protein-coding

Cancer type

Abstract

Alveolar rhabdomyosarcoma;muscular

Human cancer cells frequently harbor chromosomal translocations that create chimeric fusion genes. The t(2;13) translocation is characteristic of the pediatric muscle tumor, alveolar rhabdomyosarcoma, and produces the chimeric transcription factor, PAX3-FOXO1, that contains the DNA binding elements of PAX3and the transcriptional activation domain of FOXO1. Experiments designed to determine how PAX3-FOXO1 expression contributes to the development of muscle cell-derived tumors resulted in the discovery that the fusion protein misregulates gene expression and interrupts myogenic differentiation through a unique gain of function mechanism. These results yield new insight into how tumor-associated genetic alterations increase the likelihood of cancer formationand may lead to new therapeutic approaches.

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