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The protein tyrosine kinase JAK2 is activated in neutrophils from patients
with severe congenital neutropenia
P Rauprich, B Kasper, N Tidow and K Welte
Department of Pediatric Hematology and Oncology, Medical School Hannover,
Germany.
Severe congenital neutropenia (SCN; or Kostmann syndrome) is an autosomal
recessive disorder characterized by a maturation arrest of myelopoiesis at
the level of promyelocytes. Myeloid precursor cells from patients with SCN
require pharmacological dosages of recombinant human granulocyte
colony-stimulating factor (r-metHuG-CSF; Filgrastim; Amgen, Thousand Oaks,
CA) to differentiate to normal neutrophils. Thus, it is hypothesized that
the underlying defect responsible for SCN is based on an abnormal
G-CSF-induced signal transduction pathway. Because JAK2, a nonreceptor
tyrosine kinase, is involved in the signaling pathway of G-CSF, we examined
the expression and activity of JAK2 in neutrophils from SCN patients during
r-metHuG-CSF treatment. The immunoprecipitated JAK2 protein showed
increased tyrosine phosphorylation in neutrophils from SCN patients as
compared with that in neutrophils from healthy donors, suggesting that this
kinase is activated. In vitro kinase assays of immunoprecipitated JAK2
confirmed that neutrophils from SCN patients show an increased
autophosphorylation of JAK2 in comparison with that of neutrophils from
healthy volunteers. These findings suggest that JAK2 is activated in SCN
patients.
Volume 86,
Issue 12,
pp. 4500-4505,
12/15/1995
Copyright © 1995 by The American Society of Hematology
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