Role of PML/RARα in the pathogenesis of APL

The t(15;17) chromosomal translocation in acute promyelocytic leukemia (APL) results in the expression of the promyelocytic leukemia/retinoic acid receptor alpha (PML/RARα) fusion protein. This single gene rearrangement makes APL a unique malignancy because its oncogenic effect can be overcome by retinoic acid treatment. PML/RARα-induced leukemogenesis is a multistep process that extends beyond disruption of PML and RARα functions and requires cooperating events.

Section editor:John Tisdale – National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, USA