Oncogenes and tumor suppressors in the molecular pathogenesis of acute promyelocytic leukemia Journal Article
| Author: | Pandolfi, P. P. |
| Article Title: | Oncogenes and tumor suppressors in the molecular pathogenesis of acute promyelocytic leukemia |
| Abstract: | Acute promyelocytic leukemia (APL) is associated with reciprocal 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 vast majority of APL cases, and in a few cases to the PLZF, NPM, NuMA and Stat5b genes, respectively, leading to the generation of RARα-X and X-RARα fusion genes. Both fusion proteins can exert oncogenic functions through their ability to interfere with the activities of X and RARα proteins. Here, it will be discussed in detail how an extensive biochemical analysis as well as a systematic in vivo genetic approach in the mouse has allowed the definition of the multiple oncogenic activities of PML-RARα, and how it has become apparent that this oncoprotein is able to impair RARα at the transcription level and the tumor suppressive function of the PML protein. |
| Keywords: | controlled study; oncoprotein; human cell; mutation; pathogenesis; review; nonhuman; genetic analysis; animal cell; mouse; animals; mice; cell survival; cell division; apoptosis; models, biological; animal experiment; animal model; gene function; genetic transcription; in vivo study; cell differentiation; transcription, genetic; animalia; mice, transgenic; cloning, molecular; cancer inhibition; oncogene; leukemia, promyelocytic, acute; acute myeloblastic leukemia; gene fusion; hematopoietic stem cells; translocation, genetic; chromosome 17; gene activity; reciprocal chromosome translocation; chromosomes, human, pair 17; retinoic acid receptor; stat5b protein; receptors, retinoic acid; genes, dominant; receptor subunit; humans; human; priority journal |
| Journal Title: | Human Molecular Genetics |
| Volume: | 10 |
| Issue: | 7 |
| ISSN: | 0964-6906 |
| Publisher: | Oxford University Press |
| Date Published: | 2001-04-01 |
| Start Page: | 769 |
| End Page: | 775 |
| Language: | English |
| PUBMED: | 11257111 |
| PROVIDER: | scopus |
| DOI: | 10.1093/hmg/10.7.769 |
| DOI/URL: | |
| Notes: | Export Date: 21 May 2015 -- Source: Scopus |
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