title: [Role of PDGF/PDGFR Pathway in Essential Thrombocythemia and Its Action Mechanism].
abstract: OBJECTIVE
  To study the role of PDGF/PDGFR in essential thrombocythemia (ET) by investigating the expression of PDGF-BB in bone marrow and the expression of PDGFR-β in bone marrow cells of patients with ET and explore the new target for treating ET patients through inhibiting the PDGFR of megakaryocytes.
  
  
  METHODS
  The expression level of PDGF-BB in bone marrow of ET patients and normal controls were assayed by using ELISA, the expression level of PDGFR-β (CD140) in bone marrow of ET patients and normal controls were detected by using flow cytometry, the effect of PDGF-BB in JAK2/STAT3 and PI3K/AKT pathway was detected by using flow cytometry or Werstern blot, and the effect of imatinib on the megakaryopoiesis of PDGF was observed.
  
  
  RESULTS
  The expression level of PDGF-BB in bone marrow of ET patients was significantly higher than that in normal controls; the expression level of PDGFR-β in bone marrow of ET patients was significantly higher than that in nornal controls; PDGF-BB could activate JAK2/STAT3 and PI3K/AKT pathway of megakaryocytes, while the imatinib could block the effect of PDGF-BB on megakaryocyte.
  
  
  CONCLUSIONS
  The elevated PDGF-BB and PDGFR-β may be involved in ET, and the physiopathologic mechanism is that the elevated PDGF-BB activates PDGFR with subsequent activation of the JAK2/STAT3 and PI3K/AKT pathways, stimulating megakaryopoiesis. Imatinib may have a therapeutical effect on ET via blocking of PDGFR.
authors.affiliation: Department of Hematology, South Hospital of Southern Medical University, Guangzhou 510515, Guangdong Province, China.
authors.forename: Li-Xia
authors.initials: LX
authors.lastname: Zhou
authors.affiliation: Department of Hematology, South Hospital of Southern Medical University, Guangzhou 510515, Guangdong Province, China.
authors.forename: En-Yu
authors.initials: EY
authors.lastname: Liang
authors.affiliation: Department of Hematology, South Hospital of Southern Medical University, Guangzhou 510515, Guangdong Province, China . E-mail: jieyu.ye@gmail.com.
authors.forename: Jie-Yu
authors.initials: JY
authors.lastname: Ye
authors.affiliation: Department of Pediatrics, South Hospital of Southern Medical University, Guangzhou 510515, Guangdong Province, China. E-mail: yangm1091@126.com.
authors.forename: Mo
authors.initials: M
authors.lastname: Yang
mesh_terms: D000077214:Becaplermin; D001853:Bone Marrow / Q000378:metabolism*; D016022:Case-Control Studies; D006801:Humans; D008533:Megakaryocytes / Q000378:metabolism; D019869:Phosphatidylinositol 3-Kinases; D020574:Proto-Oncogene Proteins c-sis / Q000378:metabolism*; D020797:Receptor, Platelet-Derived Growth Factor beta / Q000378:metabolism*; D015398:Signal Transduction; D013920:Thrombocythemia, Essential / Q000378:metabolism*; D034061:Thrombopoiesis
chemical_list: D020574:Proto-Oncogene Proteins c-sis; D000077214:Becaplermin; D019869:Phosphatidylinositol 3-Kinases; D020797:Receptor, Platelet-Derived Growth Factor beta