PML nuclear body biogenesis and oligomerization-driven leukemogenesis

Yuwen Li; Xiaodan Ma; Guoyu Meng

doi:10.1097/BS9.0000000000000034

PML nuclear body biogenesis and oligomerization-driven leukemogenesis

Blood Sci. 2020 Jan 16;2(1):7-10. doi: 10.1097/BS9.0000000000000034. eCollection 2020 Jan.

Authors

Yuwen Li¹, Xiaodan Ma¹, Guoyu Meng¹

Affiliation

¹ State Key Laboratory of Medical Genomics, Shanghai Institute of Hematology, Rui-Jin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, China.

Abstract

PML nuclear bodies (NBs), which are increasingly recognized as the central hub of many cellular signaling events, are superassembled spherical complexes with diameters of 0.1-2 μm. Recent studies reveal that RING tetramerization and B1-box polymerization are key factors to the overall PML NBs assembly. The productive RBCC oligomerization allows subsequent PML biogenesis steps, including the PML auto-sumoylation and partners recruitment via SUMO-SIM interactions. In promyelocytic leukemia, the oncoprotein PML/RARα (P/R) inhibits PML NBs assembly and leads to a full-fledged leukemogenesis. In this review, we review the recent progress in PML and acute promyelocytic leukemia fields, highlighting the protein oligomerization as an important direction of future targeted therapy.

Keywords: Leukemogenesis; Oligomerization; PML nuclear body; PML/RARα.

Publication types

Review