|
|
 Previous Article | Table of Contents | Next Article 
Expression of receptors for granulocyte colony-stimulating factor on
neutrophils from patients with severe congenital neutropenia and cyclic
neutropenia
U Kyas, T Pietsch and K Welte
Department of Pediatric Hematology and Oncology, Children's Clinic, Medical
School Hannover, Germany.
We studied granulocyte colony-stimulating factor (G-CSF) binding sites on
neutrophils from patients with severe congenital neutropenia (SCN;
Kostmann-syndrome) and cyclic neutropenia (CN) during treatment with
recombinant human (rh) G-CSF. G-CSF receptor expression was measured by
scatchard analysis. Neutrophils from six healthy controls expressed between
480 and 1,210 binding sites per cell, whereas neutrophils from five SCN
patients expressed increased numbers of G-CSF binding sites ranging between
2,100 and 3,900 per cell. Neutrophils from four patients with CN expressed
350 to 1,600 binding sites per cell. The affinity of rhG-CSF to its
receptor was similar in patients and controls. These data suggest that SCN
patients and CN patients are not defective in G-CSF receptor expression as
judged by the numbers of G- CSF binding sites and binding affinity;
however, we cannot exclude defects in parts of the G-CSF receptor that may
be involved in the signal transduction pathway.
Volume 79,
Issue 5,
pp. 1144-1147,
03/01/1992
Copyright © 1992 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
N. Berliner
Lessons from congenital neutropenia: 50 years of progress in understanding myelopoiesis
Blood,
June 15, 2008;
111(12):
5427 - 5432.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Germeshausen, M. Ballmaier, and K. Welte
Incidence of CSF3R mutations in severe congenital neutropenia and relevance for leukemogenesis: results of a long-term survey
Blood,
January 1, 2007;
109(1):
93 - 99.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. Kawaguchi, M. Kobayashi, K. Nakamura, N. Konishi, S.-i. Miyagawa, T. Sato, H. Toyoda, Y. Komada, S. Kojima, Y. Todoroki, et al.
Dysregulation of transcriptions in primary granule constituents during myeloid proliferation and differentiation in patients with severe congenital neutropenia
J. Leukoc. Biol.,
February 1, 2003;
73(2):
225 - 234.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. Konishi, M. Kobayashi, S.-i. Miyagawa, T. Sato, O. Katoh, and K. Ueda
Defective Proliferation of Primitive Myeloid Progenitor Cells in Patients With Severe Congenital Neutropenia
Blood,
December 15, 1999;
94(12):
4077 - 4083.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. G. Hunter and B. R. Avalos
Deletion of a Critical Internalization Domain in the G-CSFR in Acute Myelogenous Leukemia Preceded by Severe Congenital Neutropenia
Blood,
January 15, 1999;
93(2):
440 - 446.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. Tidow, C. Pilz, B. Teichmann, A. Muller-Brechlin, M. Germeshausen, B. Kasper, P. Rauprich, K.-W. Sykora, and K. Welte
Clinical Relevance of Point Mutations in the Cytoplasmic Domain of the Granulocyte Colony-Stimulating Factor Receptor Gene in Patients With Severe Congenital Neutropenia
Blood,
April 1, 1997;
89(7):
2369 - 2375.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. K. Souid
Congenital Cyclic Neutropenia
Clinical Pediatrics,
March 1, 1995;
34(3):
151 - 155.
[PDF]
|
|
|
|
|
