Retinoic Acid Receptor α (RARα) Mutations in Human Leukemia

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Abstract
The retinoic acid receptor α (RARα) plays a central role in the biology of the myeloid cellular compartment. Chromosomal translocations involving the RARα locus probably represent the malignant initiating events in acute promyelocytic leukemia (APL). Recent studies that identify novel interactions between RARα and the nuclear receptor co-activators and co-repressors. new functions of the oncogenic RARα fusion proteins and their catabolism in retinoic acid-induced differentiation, and the availability of new transgenic mice models have provided important insights into our understanding of the mechanisms by which mutant forms of RARα can be implicated in the development of leukemia. Novel alterations of the RARα gene identified in hematopoietic malignant disorders other than APL, such as myelody-splastic syndromes, non-APL acute myeloid leukemias and B-chronic lymphocytic leukemias, suggest that disruption of the RARα gene might predispose to myeloid and lymphoid disorders.