USP11 regulates PML stability to control Notch-induced malignancy in brain tumours

Nat Commun. 2014:5:3214. doi: 10.1038/ncomms4214.

Abstract

The promyelocytic leukaemia (PML) protein controls multiple tumour suppressive functions and is downregulated in diverse types of human cancers through incompletely characterized post-translational mechanisms. Here we identify USP11 as a PML regulator by RNAi screening. USP11 deubiquitinates and stabilizes PML, thereby counteracting the functions of PML ubiquitin ligases RNF4 and the KLHL20-Cul3 (Cullin 3)-Roc1 complex. We find that USP11 is transcriptionally repressed through a Notch/Hey1-dependent mechanism, leading to PML destabilization. In human glioma, Hey1 upregulation correlates with USP11 and PML downregulation and with high-grade malignancy. The Notch/Hey1-induced downregulation of USP11 and PML not only confers multiple malignant characteristics of aggressive glioma, including proliferation, invasiveness and tumour growth in an orthotopic mouse model, but also potentiates self-renewal, tumour-forming capacity and therapeutic resistance of patient-derived glioma-initiating cells. Our study uncovers a PML degradation mechanism through Notch/Hey1-induced repression of the PML deubiquitinase USP11 and suggests an important role for this pathway in brain tumour pathogenesis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Basic Helix-Loop-Helix Transcription Factors / metabolism*
  • Brain Neoplasms / metabolism*
  • Cell Cycle Proteins / metabolism*
  • Gene Expression Regulation, Neoplastic
  • Glioblastoma / metabolism*
  • HeLa Cells
  • Humans
  • Nuclear Proteins / metabolism*
  • Promyelocytic Leukemia Protein
  • Receptors, Notch / metabolism*
  • Thiolester Hydrolases / metabolism*
  • Transcription Factors / metabolism*
  • Tumor Suppressor Proteins / metabolism*
  • Ubiquitin-Protein Ligases / metabolism

Substances

  • Basic Helix-Loop-Helix Transcription Factors
  • Cell Cycle Proteins
  • HEY1 protein, human
  • Nuclear Proteins
  • Promyelocytic Leukemia Protein
  • Receptors, Notch
  • Transcription Factors
  • Tumor Suppressor Proteins
  • USP11 protein, human
  • PML protein, human
  • Ubiquitin-Protein Ligases
  • Thiolester Hydrolases