JAK2V617F activates Lu/BCAM-mediated red cell adhesion in polycythemia vera through an EpoR-independent Rap1/Akt pathway

Blood. 2013 Jan 24;121(4):658-65. doi: 10.1182/blood-2012-07-440487. Epub 2012 Nov 16.

Abstract

Polycythemia vera (PV) is characterized by an increased RBC mass, spontaneous erythroid colony formation, and the JAK2V617F mutation. PV is associated with a high risk of mesenteric and cerebral thrombosis. PV RBC adhesion to endothelial laminin is increased and mediated by phosphorylated erythroid Lu/BCAM. In the present work, we investigated the mechanism responsible for Lu/BCAM phosphorylation in the presence of JAK2V617F using HEL and BaF3 cell lines as well as RBCs from patients with PV. High levels of Rap1-GTP were found in HEL and BaF3 cells expressing JAK2V617F compared with BaF3 cells with wild-type JAK2. This finding was associated with increased Akt activity, Lu/BCAM phosphorylation, and cell adhesion to laminin that were inhibited by the dominant-negative Rap1S17N or by the specific Rap1 inhibitor GGTI-298. Surprisingly, knocking-down EpoR in HEL cells did not alter Akt activity or cell adhesion to laminin. Our findings reveal a novel EpoR-independent Rap1/Akt signaling pathway that is activated by JAK2V617F in circulating PV RBCs and responsible for Lu/BCAM activation. This new characteristic of JAK2V617F could play a critical role in initiating abnormal interactions among circulating and endothelial cells in patients with PV.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Animals
  • Cell Adhesion / genetics
  • Cell Adhesion Molecules / genetics
  • Cell Adhesion Molecules / metabolism*
  • Cell Line
  • Erythrocytes / metabolism*
  • Female
  • Humans
  • Janus Kinase 2 / genetics
  • Janus Kinase 2 / metabolism*
  • Laminin / metabolism
  • Lutheran Blood-Group System / genetics
  • Lutheran Blood-Group System / metabolism*
  • Male
  • Mice
  • Middle Aged
  • Phosphorylation
  • Polycythemia Vera / genetics
  • Polycythemia Vera / metabolism*
  • Proto-Oncogene Proteins c-akt / metabolism*
  • Receptors, Erythropoietin / metabolism*
  • Signal Transduction
  • rap1 GTP-Binding Proteins / metabolism*

Substances

  • BCAM protein, human
  • Cell Adhesion Molecules
  • Laminin
  • Lutheran Blood-Group System
  • Receptors, Erythropoietin
  • Janus Kinase 2
  • Proto-Oncogene Proteins c-akt
  • rap1 GTP-Binding Proteins