The theory of APL revisited.
Acute promyelocytic leukemia (APL) is associated with reciprocal and balanced chromosomal translocations always involving the retinoic acid receptor alpha (RARa) gene on chromosome 17 and variable partner genes (X genes) on distinct chromosomes. RARalpha fuses to the PML gene in the majority of APL cases, and in a few cases to the PLZF, NPM, NuMA and STAT5b genes. As a consequence, X-RARalpha and RARalpha-X fusion genes are generated encoding aberrant chimeric proteins that exert critical oncogenic functions. Here we will integrate some of the most recent findings in APL research in a unified model and discuss some of the outstanding questions that remain to be addressed.
Scaglioni
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Cancer Biology and Genetics Program, Memorial Sloan-Kettering Cancer Center, Sloan-Kettering Institute, 1275 York Avenue, NY, New York 10021, USA.
Pandolfi
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D000818:Animals; D002471:Cell Transformation, Neoplastic; D004195:Disease Models, Animal; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000235:genetics / Q000503:physiopathology*; D051379:Mice; D015514:Oncogene Proteins, Fusion / Q000378:metabolism; D018168:Receptors, Retinoic Acid / Q000235:genetics / Q000378:metabolism; D000072482:Retinoic Acid Receptor alpha; D014178:Translocation, Genetic
D015514:Oncogene Proteins, Fusion; C000607071:RARA protein, human; C000607072:Rara protein, mouse; D018168:Receptors, Retinoic Acid; D000072482:Retinoic Acid Receptor alpha