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In vivo analysis of the molecular genetics of acute promyelocytic leukemia

Oncogene volume 20, pages 5726–5735 (2001)Cite this article

Abstract

Acute promyelocytic leukemia (APL) is a distinct and paradigmatic subtype of myeloid leukemia associated with reciprocal chromosomal translocations always involving the Retinoic Acid Receptorα (RARα) gene on chromosome 17 and variable partner genes (X genes) on different chromosomes. As a consequence of these translocations X-RARα and RARα-X fusion genes are generated. RARα fuses to the PML gene in the vast majority of APL cases, and in a few cases to the PLZF, NPM, NuMA and STAT5b genes respectively. In the last few years, the functions of these aberrant fusion proteins and of the normal gene products involved in these translocations have been extensively characterized in vivo in transgenic and KO animal models. Here we will review the important conclusions, the novel questions and paradoxes that stem from this analysis.

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Acknowledgements

This work is supported by the NCI, the De Witt Wallace Fund for Memorial Sloan-Kettering Cancer Center, the Mouse Model of Human Cancer Consortium (MMHCC) and NIH Grants to PP Pandolfi. We would like to thank the Lymphoma & Leukemia Society of which PP Pandolfi is a Scholar and Nilda Lena for assistance in the preparation of this manuscript. Finally, we are obviously indebted with all the past and present members of the laboratory of Molecular and Developmental Biology (MADB) lab at Memorial Sloan-Kettering Cancer Center, working on APL and related subjects.

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  1. Department of Pathology, Sloan-Kettering Division, Molecular Biology Program, Memorial Sloan-Kettering Cancer Center, Graduate School of Medical Sciences, Cornell University, 1275 York Avenue, New York, NY 10021, USA

    Pier Paolo Pandolfi

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Correspondence to Pier Paolo Pandolfi.

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Pandolfi, P. In vivo analysis of the molecular genetics of acute promyelocytic leukemia. Oncogene 20, 5726–5735 (2001). https://doi.org/10.1038/sj.onc.1204600

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