A role for PML, and the nuclear body in genomic stability

Sue Zhong, Hu Peng, Tian Zhang Ye, Rodica Stan, Nathan A. Ellis, Pier Paolo Pandolfi

Research output: Contribution to journalArticlepeer-review

199 Scopus citations


The PML gene of acute promyelocytic leukemia (APL) encodes a cell-growth and tumour suppressor. PML localizes to discrete nuclear bodies (NBs) that are disrupted in APL cells. The Bloom syndrome gene BLM encodes a RecQ DNA helicase, whose absence from the cell results in genomic instability epitomized by high levels of sister-chromatid exchange (SCE) and cancer predisposition. We show here that BLM colocalizes with PML to the NB. In cells from persons with Bloom syndrome the localization of PML is unperturbed, whereas in APL cells carrying the PML-RARα oncoprotein, both PML and BLM are delocalized from the NB into microspeckled nuclear regions. Treatment with retinoic acid (RA) induces the relocalization of both proteins to the NE. In primary PML-/- cells, BLM fails to accumulate in the NB. Strikingly, in PML-/- cells the frequency of SCEs is increased relative to PML+/+ cells. These data demonstrate that BLM is a constituent of the NB and that PML is required for its accumulation in these nuclear domains and for the normal function of BLM. Thus, our findings suggest a role for BLM in APL pathogenesis and implicate the PML NB in the maintenance of genomic stability.

Original languageEnglish (US)
Pages (from-to)7941-7947
Number of pages7
Issue number56
StatePublished - Dec 23 1999


  • APL
  • BLM
  • Bloom syndrome
  • Nuclear body
  • PML
  • Sister-chromatid exchange

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Cancer Research


Dive into the research topics of 'A role for PML, and the nuclear body in genomic stability'. Together they form a unique fingerprint.

Cite this