de Thé H
CNRS UPR, 43, Hôpital Saint-Louis, Paris.
C R Seances Soc Biol Fil. 1995;189(4):503-14.
Acute promyelocytic leukemia is a key model system in Cancer biology. Its ability to differentiate upon exposure to retinoic acid constitutes the first example of differentiation therapy. The molecular basis of leukemogenesis is the PML/RAR fusion resulting from the t(15, 17) translocation. The fusion protein likely acts through interference with the function of nuclear receptors (resulting in a differentiation block) and also through interference with PML which appear to be a growth suppressor. The exquisite sensitivity of this disease to retinoic acid represents the first example of a therapy directly targeted at a specific and causative genetic lesion.
急性早幼粒细胞白血病是癌症生物学中的一个关键模型系统。其在接触视黄酸后发生分化的能力构成了分化疗法的首个实例。白血病发生的分子基础是由t(15;17)易位导致的PML/RAR融合。该融合蛋白可能通过干扰核受体的功能（导致分化阻滞）以及干扰似乎是生长抑制因子的PML发挥作用。这种疾病对视黄酸的高度敏感性代表了直接针对特定致病基因损伤的疗法的首个实例。
