In vivo analysis of the molecular genetics of acute promyelocytic leukemia Journal Article


Author: Pandolfi, P. P.
Article Title: In vivo analysis of the molecular genetics of acute promyelocytic leukemia
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.
Keywords: leukemia; oncoprotein; review; nonhuman; mouse; animals; mice; animal model; protein binding; transcription factor; genetic transcription; transcription, genetic; transgenic mouse; animalia; mus musculus; transcription factors; leukemia, promyelocytic, acute; acute leukemia; hybrid protein; leukemogenesis; acute myeloblastic leukemia; oncogene proteins, fusion; chromosome translocation; x chromosome; translocation, genetic; models, genetic; disease models, animal; retinoic acid; chromosome 17; clinical trials; transgenic mice; arylbutyric acid derivative; trichostatin a; animal models; chromosomes, human, pair 17; retinoic acid receptor; hydroxamic acid derivative; receptors, retinoic acid; apl; cancer; humans; priority journal; ko mice
Journal Title: Oncogene
Volume: 20
Issue: 40
ISSN: 0950-9232
Publisher: Nature Publishing Group  
Date Published: 2001-09-10
Start Page: 5726
End Page: 5735
Language: English
DOI: 10.1038/sj.onc.1204600
PUBMED: 11607822
PROVIDER: scopus
DOI/URL:
Notes: Export Date: 21 May 2015 -- Source: Scopus
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors