早幼粒细胞白血病-维A酸受体融合基因的研究进展

Promyelocytic leukemia-retinoic acid receptor α fusion gene:research advances

急性早幼粒细胞白血病是急性粒细胞白血病的一个亚型。它的分子生物学特征为15号染色体上的早幼粒细胞白血病(PML)基因与17号染色体上的维A酸受体α(RARα)基因发生易位,表达PML-RARα融合蛋白。PML-RARα融合蛋白在急性早幼粒细胞白血病的发生、发展、诊断和治疗中发挥着重要作用。本文主要综述PML蛋白的结构和生物学功能,PMLRARα的结构、功能及其降解途径。

Acute promyelocytic leukemia （APL） is a distinctive subtype of acute myeloid leukemia with a distinct biology and clinical presentation. Its molecular biology characteristic is a aberrant chromosomal transloeation of the promyelocytie leukemia （PML） gene on chromosome 15 and the retinoie acid receptor or（RARer） geue on chromosome 17. This translocation generates PML- RARα fusion protein, which plays an important role in the genesis, development, diagnosis and therapy of APL. The PML protein has a close relationship with PML-RARα fusion gene. This article mainly summarizes the character, the function of PML protein and the degradation pathway of PML-RARα.