SIRT3 overexpression and epigenetic silencing of catalase regulate ROS accumulation in CLL cells activating AXL signaling axis

Blood Cancer J. 2021 May 17;11(5):93. doi: 10.1038/s41408-021-00484-6.

Abstract

Mitochondrial metabolism is the key source for abundant ROS in chronic lymphocytic leukemia (CLL) cells. Here, we detected significantly lower superoxide anion (O2-) levels with increased accumulation of hydrogen peroxide (H2O2) in CLL cells vs. normal B-cells. Further analysis indicated that mitochondrial superoxide dismutase (SOD)2, which converts O2- into H2O2 remained deacetylated in CLL cells due to SIRT3 overexpression resulting its constitutive activation. In addition, catalase expression was also reduced in CLL cells suggesting impairment of H2O2-conversion into water and O2 which may cause H2O2-accumulation. Importantly, we identified two CpG-islands in the catalase promoter and discovered that while the distal CpG-island (-3619 to -3765) remained methylated in both normal B-cells and CLL cells, variable degrees of methylation were discernible in the proximal CpG-island (-174 to -332) only in CLL cells. Finally, treatment of CLL cells with a demethylating agent increased catalase mRNA levels. Functionally, ROS accumulation in CLL cells activated the AXL survival axis while upregulated SIRT3, suggesting that CLL cells rapidly remove highly reactive O2- to avoid its cytotoxic effect but maintain increased H2O2-level to promote cell survival. Therefore, abrogation of aberrantly activated cell survival pathways using antioxidants can be an effective intervention in CLL therapy in combination with conventional agents.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Axl Receptor Tyrosine Kinase
  • Catalase / genetics*
  • Catalase / metabolism
  • Female
  • Gene Expression Regulation, Leukemic
  • Gene Silencing
  • Humans
  • Leukemia, Lymphocytic, Chronic, B-Cell / genetics*
  • Leukemia, Lymphocytic, Chronic, B-Cell / metabolism
  • Male
  • Middle Aged
  • Proto-Oncogene Proteins / metabolism*
  • Reactive Oxygen Species / metabolism*
  • Receptor Protein-Tyrosine Kinases / metabolism*
  • Signal Transduction*
  • Sirtuin 3 / genetics*
  • Sirtuin 3 / metabolism
  • Tumor Cells, Cultured
  • Up-Regulation

Substances

  • Proto-Oncogene Proteins
  • Reactive Oxygen Species
  • CAT protein, human
  • Catalase
  • Receptor Protein-Tyrosine Kinases
  • SIRT3 protein, human
  • Sirtuin 3
  • Axl Receptor Tyrosine Kinase
  • AXL protein, human