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The theory of APL
F Piazza1, C Gurrieri, P P Pandolfi
1Molecular Biology Program, Department of Pathology, Memorial Sloan-Kettering Cancer Center, Sloan-Kettering Division, Graduate School of Medical Sciences, Cornell University, 1275 York Avenue, New York, New York, NY 10021, USA.
Oncogene
|November 13, 2001
Summary
Acute promyelocytic leukemia (APL) involves chromosomal translocations fusing the Retinoic Acid Receptor alpha (RARalpha) gene with other genes. Mouse models reveal how these fusion genes cause APL by creating aberrant proteins.
Area of Science:
- Hematology
- Molecular Biology
- Genetics
Background:
- Acute promyelocytic leukemia (APL) is characterized by specific chromosomal translocations.
- These translocations invariably involve the Retinoic Acid Receptor alpha (RARalpha) gene.
Purpose of the Study:
- To review findings from a decade of in vivo mouse studies on APL pathogenesis.
- To explore open questions regarding the role of fusion genes in APL development.
Main Methods:
- Analysis of transgenic mouse models.
- Analysis of knock-out mouse models.
- In vivo studies of APL pathogenesis.
Main Results:
- Reciprocal translocations create fusion genes (e.g., PML-RARalpha).
- Fusion proteins generated by these genes interfere with normal cellular functions.
- Mouse models recapitulate key aspects of human APL.
Conclusions:
- In vivo mouse models are crucial for understanding APL pathogenesis.
- Aberrant fusion proteins are central to the development of APL.
- Further research is needed to address open questions in APL biology.