KSHV-induced ligand mediated activation of PDGF receptor-alpha drives Kaposi's sarcomagenesis

PLoS Pathog. 2018 Jul 9;14(7):e1007175. doi: 10.1371/journal.ppat.1007175. eCollection 2018 Jul.

Abstract

Kaposi's sarcoma (KS) herpesvirus (KSHV) causes KS, an angiogenic AIDS-associated spindle-cell neoplasm, by activating host oncogenic signaling cascades through autocrine and paracrine mechanisms. Tyrosine kinase receptor (RTK) proteomic arrays, identified PDGF receptor-alpha (PDGFRA) as the predominantly-activated RTK in KSHV-induced mouse KS-tumors. We show that: 1) KSHV lytic replication and the vGPCR can activate PDGFRA through upregulation of its ligands PDGFA/B, which increase c-myc, VEGF and KSHV gene expression in infected cells 2) KSHV infected spindle cells of most AIDS-KS lesions display robust phospho-PDGFRA staining 3) blocking PDGFRA-signaling with N-acetyl-cysteine, RTK-inhibitors Imatinib and Sunitinib, or dominant-negative PDGFRA inhibits tumorigenesis 4) PDGFRA D842V activating-mutation confers resistance to Imatinib in mouse-KS tumorigenesis. Our data show that KSHV usurps sarcomagenic PDGFRA signaling to drive KS. This and the fact that PDGFRA drives non-viral sarcomas highlights the importance for KSHV-induced ligand-mediated activation of PDGFRA in KS sarcomagenesis and shows that this oncogenic axis could be successfully blocked to impede KS tumor growth.

Publication types

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

MeSH terms

  • Animals
  • Carcinogenesis / metabolism*
  • Herpesvirus 8, Human / pathogenicity*
  • Humans
  • Mice
  • Mice, Nude
  • Platelet-Derived Growth Factor / metabolism
  • Proto-Oncogene Proteins c-sis / metabolism
  • Receptor, Platelet-Derived Growth Factor alpha / metabolism*
  • Sarcoma, Kaposi / metabolism
  • Sarcoma, Kaposi / virology*
  • Signal Transduction

Substances

  • Platelet-Derived Growth Factor
  • Proto-Oncogene Proteins c-sis
  • platelet-derived growth factor A
  • Receptor, Platelet-Derived Growth Factor alpha