H. Miyoshi et al., THE T(8-21) TRANSLOCATION IN ACUTE MYELOID-LEUKEMIA RESULTS IN PRODUCTION OF AN AML1-MTG8 FUSION TRANSCRIPT, EMBO journal, 12(7), 1993, pp. 2715-2721
The t(8;21) translocation is one of the most frequent chromosome abnor
malities in acute myeloid leukemia. It has been shown that the t(8;21)
breakpoints on chromosome 21 cluster within a single specific intron
of the AML1 gene, which is highly homologous to the Drosophila segment
ation gene runt. Here we report that this translocation juxtaposes the
AML1 gene with a novel gene, named MTG8, on chromosome 8, resulting i
n the synthesis of an AML1-MTG8 fusion transcript. The fusion protein
predicted by the AML1-MTG8 transcript consists of the runt homology re
gion of AML1 and the most part of MTG8, which contains putative zinc r
inger DNA binding motifs and proline-rich regions constituting a chara
cteristic feature of transcription factors. The MTG8 gene is not expre
ssed in normal hematopoietic cells, whereas AML1 is expressed at high
levels. Our results indicate that the production of chimeric AML1-MTG8
protein, probably a chimeric transcription factor, may contribute to
myeloid leukemogenesis.