Scaglioni P P, Pandolfi P P
Cancer Biology and Genetics Program, Memorial Sloan-Kettering Cancer Center, Sloan-Kettering Institute, 1275 York Avenue, NY, New York 10021, USA.
Curr Top Microbiol Immunol. 2007;313:85-100. doi: 10.1007/978-3-540-34594-7_6.
Acute promyelocytic leukemia (APL) is associated with reciprocal and balanced chromosomal translocations always involving the retinoic acid receptor alpha (RARa) gene on chromosome 17 and variable partner genes (X genes) on distinct chromosomes. RARalpha fuses to the PML gene in the majority of APL cases, and in a few cases to the PLZF, NPM, NuMA and STAT5b genes. As a consequence, X-RARalpha and RARalpha-X fusion genes are generated encoding aberrant chimeric proteins that exert critical oncogenic functions. Here we will integrate some of the most recent findings in APL research in a unified model and discuss some of the outstanding questions that remain to be addressed.
急性早幼粒细胞白血病（APL）与相互平衡的染色体易位相关，这些易位总是涉及17号染色体上的维甲酸受体α（RARα）基因以及不同染色体上的可变伙伴基因（X基因）。在大多数APL病例中，RARα与PML基因融合，少数情况下与PLZF、NPM、NuMA和STAT5b基因融合。因此，会产生X-RARα和RARα-X融合基因，它们编码具有关键致癌功能的异常嵌合蛋白。在此，我们将把APL研究中的一些最新发现整合到一个统一模型中，并讨论一些有待解决的突出问题。
