A role for MALT1 activity in Kaposi's sarcoma-associated herpes virus latency and growth of primary effusion lymphoma

Leukemia. 2017 Mar;31(3):614-624. doi: 10.1038/leu.2016.239. Epub 2016 Aug 19.

Abstract

Primary effusion lymphoma (PEL) is an incurable malignancy that develops in immunodeficient patients as a consequence of latent infection of B-cells with Kaposi's sarcoma-associated herpes virus (KSHV). Malignant growth of KSHV-infected B cells requires the activity of the transcription factor nuclear factor (NF)-κB, which controls maintenance of viral latency and suppression of the viral lytic program. Here we show that the KSHV proteins K13 and K15 promote NF-κB activation via the protease mucosa-associated lymphoid tissue lymphoma translocation protein-1 (MALT1), a key driver of NF-κB activation in lymphocytes. Inhibition of the MALT1 protease activity induced a switch from the latent to the lytic stage of viral infection, and led to reduced growth and survival of PEL cell lines in vitro and in a xenograft model. These results demonstrate a key role for the proteolytic activity of MALT1 in PEL, and provide a rationale for the pharmacological targeting of MALT1 in PEL therapy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Biomarkers
  • Caspases / genetics
  • Caspases / metabolism*
  • Cell Line
  • Cell Survival / genetics
  • Disease Models, Animal
  • Enzyme Activation
  • Flow Cytometry
  • Gene Silencing
  • Herpesviridae Infections / complications*
  • Herpesviridae Infections / virology*
  • Herpesvirus 8, Human / physiology*
  • Host-Pathogen Interactions
  • Humans
  • Lymphoma, Primary Effusion / etiology*
  • Lymphoma, Primary Effusion / pathology*
  • Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein
  • NF-kappa B / metabolism
  • Neoplasm Proteins / genetics
  • Neoplasm Proteins / metabolism*
  • Protein Binding
  • Viral Proteins / metabolism
  • Virus Activation
  • Virus Latency*
  • Xenograft Model Antitumor Assays

Substances

  • Biomarkers
  • NF-kappa B
  • Neoplasm Proteins
  • Viral Proteins
  • Caspases
  • MALT1 protein, human
  • Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein