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

M De Grandis, M Cambot, MP Wautier… - Blood, The Journal …, 2013 - ashpublications.org
M De Grandis, M Cambot, MP Wautier, B Cassinat, C Chomienne, Y Colin, JL Wautier…
Blood, The Journal of the American Society of Hematology, 2013ashpublications.org
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 …
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.
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