EBNA3B-deficient EBV promotes B cell lymphomagenesis in humanized mice and is found in human tumors

J Clin Invest. 2012 Apr;122(4):1487-502. doi: 10.1172/JCI58092. Epub 2012 Mar 12.

Abstract

Epstein-Barr virus (EBV) persistently infects more than 90% of the human population and is etiologically linked to several B cell malignancies, including Burkitt lymphoma (BL), Hodgkin lymphoma (HL), and diffuse large B cell lymphoma (DLBCL). Despite its growth transforming properties, most immune-competent individuals control EBV infection throughout their lives. EBV encodes various oncogenes, and of the 6 latency-associated EBV-encoded nuclear antigens, only EBNA3B is completely dispensable for B cell transformation in vitro. Here, we report that infection with EBV lacking EBNA3B leads to aggressive, immune-evading monomorphic DLBCL-like tumors in NOD/SCID/γc-/- mice with reconstituted human immune system components. Infection with EBNA3B-knockout EBV (EBNA3BKO) induced expansion of EBV-specific T cells that failed to infiltrate the tumors. EBNA3BKO-infected B cells expanded more rapidly and secreted less T cell-chemoattractant CXCL10, reducing T cell recruitment in vitro and T cell-mediated killing in vivo. B cell lines from 2 EBV-positive human lymphomas encoding truncated EBNA3B exhibited gene expression profiles and phenotypic characteristics similar to those of tumor-derived lines from the humanized mice, including reduced CXCL10 secretion. Screening EBV-positive DLBCL, HL, and BL human samples identified additional EBNA3B mutations. Thus, EBNA3B is a virus-encoded tumor suppressor whose inactivation promotes immune evasion and virus-driven lymphomagenesis.

Publication types

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

MeSH terms

  • Animals
  • Cell Line, Transformed / transplantation
  • Cell Line, Transformed / virology
  • Cell Transformation, Viral / genetics*
  • Chemokine CXCL10 / biosynthesis
  • Chemokine CXCL10 / deficiency
  • Chemokine CXCL10 / genetics
  • Chimera
  • DNA Mutational Analysis
  • Epstein-Barr Virus Infections / genetics
  • Epstein-Barr Virus Infections / virology*
  • Epstein-Barr Virus Nuclear Antigens / genetics
  • Epstein-Barr Virus Nuclear Antigens / physiology*
  • Gene Deletion
  • Genes, Tumor Suppressor*
  • Genes, Viral*
  • Hematopoietic Stem Cell Transplantation
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / pathogenicity
  • Herpesvirus 4, Human / physiology*
  • Humans
  • Interferon-gamma / deficiency
  • Interferon-gamma / genetics
  • Lymphoma, B-Cell / genetics
  • Lymphoma, B-Cell / virology*
  • Lymphoproliferative Disorders / genetics
  • Lymphoproliferative Disorders / virology*
  • Mice
  • Mice, Inbred NOD
  • Mice, Knockout
  • Mice, SCID
  • Mutation
  • Postoperative Complications / genetics
  • Postoperative Complications / virology*
  • Transplantation, Heterologous
  • Tumor Suppressor Proteins / genetics
  • Tumor Suppressor Proteins / physiology*
  • Tumor Virus Infections / genetics
  • Tumor Virus Infections / virology*

Substances

  • CXCL10 protein, human
  • Chemokine CXCL10
  • EBNA-3B antigen
  • Epstein-Barr Virus Nuclear Antigens
  • Tumor Suppressor Proteins
  • Interferon-gamma