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Blood, Vol. 95 No. 9 (May 1), 2000:
pp. 2947-2953
Differential expression and regulation of GTPases (RhoA and
Rac2) and GDIs (LyGDI and RhoGDI) in neutrophils from
patients with severe congenital neutropenia
Brigitte Kasper,
Nicola Tidow,
Dirk Grothues, and
Karl Welte
Department of Pediatric Hematology/Oncology, Medical School
Hannover, Hannover, Germany.
Severe congenital neutropenia (SCN) or Kostmann syndrome is a
disorder of myelopoiesis characterized by a maturation arrest at the
stage of promyelocytes or myelocytes in bone marrow and absolute
neutrophil counts less than 200/µL in peripheral blood. Treatment of
these patients with granulocyte colony-stimulating factor (G-CSF) leads
to a significant increase in circulating neutrophils and a reduction in
infection-related events in more than 95% of the patients. To date,
little is known regarding the underlying pathomechanism of SCN.
G-CSF-induced neutrophils of patients with SCN are functionally
defective (eg, chemotaxis, superoxide anion generation, Ca++
mobilization). Two guanosine triphosphatases (GTPases), Rac2
and RhoA, were described to be involved in many neutrophil functions. The expression of these GTPases and their regulation in patients' neutrophils were of interest. This study determined that the guanosine diphosphate (GDP)-dissociation inhibitor RhoGDI is overexpressed at the
protein level in patients' neutrophils and that overexpression is a
result of G-CSF treatment. RhoA and LyGDI are expressed at similar
levels, whereas Rac2 shows a decreased expression. In addition,
association of Rac2 and RhoGDI or LyGDI is abrogated or not detectable
based on the low Rac2 expression in patients' neutrophils.
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