Mechanisms of ETV6/RUNX1-mediated gene regulation in leukemia
C Nassimbeni, G Fuka, M Morak, R Grausenburger, ER Panzer-Grümayer · Klinische Pädiatrie · 2012 · Heft 04
Bibliografische Angaben
Zusammenfassung
The t(12;21)(p13;q22) chromosomal translocation, which generates the ETV6/RUNX1 (E/R) fusion gene, is present in about 20% of childhood acute lymphoblastic leukemia (ALL). It leads to a chimeric transcription factor consisting of the N-terminal region of ETV6 and almost the entire RUNX1 protein. E/R is under the transcriptional control of the ETV6 promoter and regulates transcription via the DNA-binding RUNT homology domain of RUNX1. So far, experimental evidence suggested that E/R acts as a constitutive repressor of RUNX1 target genes by aberrantly recruiting transcriptional repressors. This notion, however, ha…