B-Cell-Specific Diversion of Glucose Carbon Utilization Reveals a Unique Vulnerability in B Cell Malignancies

Cell. 2018 Apr 5;173(2):470-484.e18. doi: 10.1016/j.cell.2018.02.048. Epub 2018 Mar 15.

Abstract

B cell activation during normal immune responses and oncogenic transformation impose increased metabolic demands on B cells and their ability to retain redox homeostasis. While the serine/threonine-protein phosphatase 2A (PP2A) was identified as a tumor suppressor in multiple types of cancer, our genetic studies revealed an essential role of PP2A in B cell tumors. Thereby, PP2A redirects glucose carbon utilization from glycolysis to the pentose phosphate pathway (PPP) to salvage oxidative stress. This unique vulnerability reflects constitutively low PPP activity in B cells and transcriptional repression of G6PD and other key PPP enzymes by the B cell transcription factors PAX5 and IKZF1. Reflecting B-cell-specific transcriptional PPP-repression, glucose carbon utilization in B cells is heavily skewed in favor of glycolysis resulting in lack of PPP-dependent antioxidant protection. These findings reveal a gatekeeper function of the PPP in a broad range of B cell malignancies that can be efficiently targeted by small molecule inhibition of PP2A and G6PD.

Keywords: B cell malignancies; G6PD; PP2A; glucose metabolism; lineage-specific vulnerability; redox homeostasis; transcriptional repression.

Publication types

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

MeSH terms

  • Animals
  • B-Lymphocytes / cytology
  • B-Lymphocytes / metabolism
  • Carbon / metabolism*
  • Cell Line, Tumor
  • Cell Survival
  • Glucose / metabolism*
  • Glucosephosphate Dehydrogenase / genetics
  • Glucosephosphate Dehydrogenase / metabolism
  • Glycolysis
  • Humans
  • Ikaros Transcription Factor / genetics
  • Ikaros Transcription Factor / metabolism
  • Mice
  • Mice, Inbred C57BL
  • Mice, Inbred NOD
  • Oxidative Stress
  • PAX5 Transcription Factor / genetics
  • PAX5 Transcription Factor / metabolism
  • Pentose Phosphate Pathway
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / metabolism
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology*
  • Protein Phosphatase 2 / deficiency
  • Protein Phosphatase 2 / genetics
  • Protein Phosphatase 2 / metabolism
  • Proto-Oncogene Proteins c-bcl-2 / metabolism
  • Transcription, Genetic

Substances

  • IKZF1 protein, human
  • PAX5 Transcription Factor
  • PAX5 protein, human
  • Proto-Oncogene Proteins c-bcl-2
  • Ikaros Transcription Factor
  • Carbon
  • Glucosephosphate Dehydrogenase
  • Protein Phosphatase 2
  • Glucose