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Featured Review
Cellscience Reviews Vol 4 No 4
ISSN 1742-8130 |
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Many paths to one disease: the role of the variant fusion proteins
NPM-RARα and NuMA-RARα in acute promyelocytic leukemia biology
Mariam Thomas 1,2*, Mahadeo A. Sukhai 1* & Suzanne Kamel-Reid 1,2,3
1 Division of Applied Molecular Oncology, Princess Margaret Hospital/the Ontario Cancer Institute, University Health Network,
2 Dept. of Medical Biophysics &
3 Dept. of Laboratory Medicine & Pathobiology, University of Toronto, Toronto, ON, Canada.
* These two authors contributed equally to this work
Received 14th April © Cellscience 2008
Acute promyelocytic leukemia (APL) is characterized at the molecular level by the presence of the X-RARα fusion gene in leukemic cells. Currently, seven X-RARα have been identified in APL patients around the world. In this review, we summarize the current understanding of the roles of NPM-RARα and NuMA-RARα within the cell. We discuss the wild-type functions of NPM, NuMA and RARα, and the potential consequences posed by the presence of the fusion proteins in the cell. We review the work conducted on the subcellular localization, protein-protein interactions and transcriptional properties of these fusion proteins. Taken together, the data we present herein suggest several additional ways by which X-RARα may affect the cellular phenotype and cause disease.
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