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The Theory of APL Revisited

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Acute Promyelocytic Leukemia

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 313))

Abstract

Acute promyelocytic leukemia (APL) is associated with reciprocal and balanced chromosomal translocations always involving the retinoic acid receptor α (RARα) gene on chromosome 17 and variable partner genes (X genes) on distinct chromosomes. RARα 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-RARα and RARα-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.

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Authors and Affiliations

  1. Cancer Biology and Genetics Program, Memorial Sloan-Kettering Cancer Center, Sloan-Kettering Institute, 1275 York Avenue, 10021, NY, New York, USA

    P. P. Scaglioni & P. P. Pandolfi

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  1. P. P. Scaglioni
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  2. P. P. Pandolfi
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Editors and Affiliations

  1. Memorial Sloan-Kettering Cancer Center, Sloan-Kettering-Institute, 1275 York Avenue, New York, New York, 10021, USA

    Pier Paolo Pandolfi MD, Ph.D.

  2. The Scripps Research Institute, 10550 North Torreye Pines Road, La Jolla, CA, 92037, USA

    Peter K. Vogt Ph.D.

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Scaglioni, P.P., Pandolfi, P.P. (2007). The Theory of APL Revisited. In: Pandolfi, P.P., Vogt, P.K. (eds) Acute Promyelocytic Leukemia. Current Topics in Microbiology and Immunology, vol 313. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-34594-7_6

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