I. Sanchezgarcia et Th. Rabbitts, TRANSCRIPTIONAL ACTIVATION BY TAL1 AND FUS-CHOP PROTEINS EXPRESSED INACUTE MALIGNANCIES AS A RESULT OF CHROMOSOMAL-ABNORMALITIES, Proceedings of the National Academy of Sciences of the United Statesof America, 91(17), 1994, pp. 7869-7873
Proteins that appear to participate in transcriptional control of gene
expression are increasingly implicated in leukemias and malignant sol
id tumors. We report here that the N-terminal domains of the proteins
TAL1 (ectopically activated in T-cell acute leukemias after chromosoma
l abnormalities caused by V-D-J recombinase error) (V, variable; D, di
versity; J, joining) and FUS-CHOP (a liposarcoma tumor-specific fusion
protein that is produced as a result of a chromosomal translocation)
can function as transcription activators of specific responsive report
er genes. The result with TAL1 pro,ides evidence that transcriptional
activation can be mediated by a gene activated by translocation in T-c
ell acute leukemias. In the case of the liposarcoma, transactivation b
y the FUS-CHOP protein occurs because the FUS transcriptional activati
on domain is added to the DNA-binding CHOP protein normally lacking su
ch activity. Therefore, the association of transcriptional activation
and DNA-binding elements is a common consequence in proteins activated
or newly created as fusion proteins after chromosomal translocations
in acute leukemias and in malignant solid tumors.