AME Transcription Factor

Synonyms

RUNX1/MDS1/EVI1; AML1/EVI-1

Definition

AME is an aggressive oncoprotein (chimeric transcription factor) associated with several types of acute myeloid leukemia (AML), myelodysplastic syndrome (MDS) and myeloproliferative disorders (MPD).

Characteristics

The legendary discovery of chromosomal translocations by Janet D. Rowley in 1972 has revolutionized leukemia research and therapy by allowing biological interrogation and classification of these disorders. Several recurring translocations have been identified and the participating genes cloned and characterized at the molecular level. One such recurring abnormality is the balanced translocation between the long arms of chromosomes 3 and 21, t(3;21)(q26;q22), originally discovered in a patient with therapy-related chronic myelogenous leukemia (CML) which is classified as an MPD.

The t(3;21) is a complicated chromosomal rearrangement that employs a mechanism of intergenic splicing to generate