Blood online
Home About Blood Authors Subscriptions Permission Advertising Public Access contact us
 

 
Advanced
Current Issue
First Edition
Archives
Submit to Blood
Search
American Society of Hematology
Meeting Abstracts
Email Alerts
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Right arrow Rights and Permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Hermans, M. H.A.
Right arrow Articles by Touw, I. P.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Hermans, M. H.A.
Right arrow Articles by Touw, I. P.
Social Bookmarking
 Add to CiteULike   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati  
What's this?

arrow to previous article Previous Article  |  Table of Contents  |  Next Article next article arrow

Perturbed Granulopoiesis in Mice With a Targeted Mutation in the Granulocyte Colony-Stimulating Factor Receptor Gene Associated With Severe Chronic Neutropenia

Mirjam H.A. Hermans, Alister C. Ward, Claudia Antonissen, Alar Karis, Bob Löwenberg, and Ivo P. Touw

From the Institute of Hematology, Daniel den Hoed Cancer Center and Erasmus University Rotterdam; and the Department of Cell Biology and Genetics, Erasmus University Rotterdam, Rotterdam, The Netherlands.

Mutations in the granulocyte colony-stimulating factor (G-CSF) receptor gene are found in a number of patients with severe chronic neutropenia predisposed to acute myeloid leukemia. These mutations result in the absence of the C-terminal domain of the G-CSF-R, a region which has been implicated in differentiation signaling. We generated mice with an equivalent mutation (gcsfr-triangle 715) by homologous and Cre-mediated recombination in embryonic stem cells. Both wt/triangle 715 and triangle 715/triangle 715 mice have significantly reduced numbers of blood neutrophils compared with their wt/wt littermates. However, under continuous G-CSF administration mutant mice develop peripheral neutrophil counts that significantly exceed those of wild-type littermates. These findings indicate that depending on G-CSF levels in mice, the triangle 715 mutation can contribute both to neutropenia and to neutrophilia.

Blood, Vol. 92 No. 1 (July 1), 1998: pp. 32-39
© 1998 by The American Society of Hematology.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati    What's this?


This article has been cited by other articles:


Home page
BloodHome page
D. C. Link, G. Kunter, Y. Kasai, Y. Zhao, T. Miner, M. D. McLellan, R. E. Ries, D. Kapur, R. Nagarajan, D. C. Dale, et al.
Distinct patterns of mutations occurring in de novo AML versus AML arising in the setting of severe congenital neutropenia
Blood, September 1, 2007; 110(5): 1648 - 1655.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
C. H. Mermel, M. L. McLemore, F. Liu, S. Pereira, J. Woloszynek, C. A. Lowell, and D. C. Link
Src family kinases are important negative regulators of G-CSF-dependent granulopoiesis
Blood, October 15, 2006; 108(8): 2562 - 2568.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
P. S. Rosenberg, B. P. Alter, A. A. Bolyard, M. A. Bonilla, L. A. Boxer, B. Cham, C. Fier, M. Freedman, G. Kannourakis, S. Kinsey, et al.
The incidence of leukemia and mortality from sepsis in patients with severe congenital neutropenia receiving long-term G-CSF therapy
Blood, June 15, 2006; 107(12): 4628 - 4635.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
A. Wolfler, S. J. Erkeland, C. Bodner, M. Valkhof, W. Renner, C. Leitner, W. Olipitz, M. Pfeilstocker, C. Tinchon, W. Emberger, et al.
A functional single-nucleotide polymorphism of the G-CSF receptor gene predisposes individuals to high-risk myelodysplastic syndrome
Blood, May 1, 2005; 105(9): 3731 - 3736.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
L. J. Druhan, J. Ai, P. Massullo, T. Kindwall-Keller, M. A. Ranalli, and B. R. Avalos
Novel mechanism of G-CSF refractoriness in patients with severe congenital neutropenia
Blood, January 15, 2005; 105(2): 584 - 591.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
G.-J. M. van de Geijn, J. Gits, L. H. J. Aarts, C. Heijmans-Antonissen, and I. P. Touw
G-CSF receptor truncations found in SCN/AML relieve SOCS3-controlled inhibition of STAT5 but leave suppression of STAT3 intact
Blood, August 1, 2004; 104(3): 667 - 674.
[Abstract] [Full Text] [PDF]


Home page
J. Leukoc. Biol.Home page
G.-J. M. van de Geijn, J. Gits, and I. P. Touw
Distinct activities of suppressor of cytokine signaling (SOCS) proteins and involvement of the SOCS box in controlling G-CSF signaling
J. Leukoc. Biol., July 1, 2004; 76(1): 237 - 244.
[Abstract] [Full Text] [PDF]


Home page
J. Biol. Chem.Home page
A. Kimura, I. Kinjyo, Y. Matsumura, H. Mori, R. Mashima, M. Harada, K. R. Chien, H. Yasukawa, and A. Yoshimura
SOCS3 Is a Physiological Negative Regulator for Granulopoiesis and Granulocyte Colony-stimulating Factor Receptor Signaling
J. Biol. Chem., February 20, 2004; 279(8): 6905 - 6910.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
L. H. J. Aarts, O. Roovers, A. C. Ward, and I. P. Touw
Receptor activation and 2 distinct COOH-terminal motifs control G-CSF receptor distribution and internalization kinetics
Blood, January 15, 2004; 103(2): 571 - 579.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
V. Santini, B. Scappini, Z. K. Indik, A. Gozzini, P. Rossi Ferrini, and A. D. Schreiber
The carboxy-terminal region of the granulocyte colony-stimulating factor receptor transduces a phagocytic signal
Blood, June 1, 2003; 101(11): 4615 - 4622.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
T. Mitsui, S. Watanabe, Y. Taniguchi, S. Hanada, Y. Ebihara, T. Sato, T. Heike, M. Mitsuyama, T. Nakahata, and K. Tsuji
Impaired neutrophil maturation in truncated murine G-CSF receptor-transgenic mice
Blood, April 15, 2003; 101(8): 2990 - 2995.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
M. H. A. Hermans, G.-J. van de Geijn, C. Antonissen, J. Gits, D. van Leeuwen, A. C. Ward, and I. P. Touw
Signaling mechanisms coupled to tyrosines in the granulocyte colony-stimulating factor receptor orchestrate G-CSF-induced expansion of myeloid progenitor cells
Blood, April 1, 2003; 101(7): 2584 - 2590.
[Abstract] [Full Text] [PDF]


Home page
J. Leukoc. Biol.Home page
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]


Home page
BloodHome page
S. J. Erkeland, M. Valkhof, C. Heijmans-Antonissen, R. Delwel, P. J. M. Valk, M. H. A. Hermans, and I. P. Touw
The gene encoding the transcriptional regulator Yin Yang 1 (YY1) is a myeloid transforming gene interfering with neutrophilic differentiation
Blood, February 1, 2003; 101(3): 1111 - 1117.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
D. S. Grenda, S. E. Johnson, J. R. Mayer, M. L. McLemore, K. F. Benson, M. Horwitz, and D. C. Link
Mice expressing a neutrophil elastase mutation derived from patients with severe congenital neutropenia have normal granulopoiesis
Blood, October 16, 2002; 100(9): 3221 - 3228.
[Abstract] [Full Text] [PDF]


Home page
J. Immunol.Home page
F. Dong, Y. Qiu, T. Yi, I. P. Touw, and A. C. Larner
The Carboxyl Terminus of the Granulocyte Colony- Stimulating Factor Receptor, Truncated in Patients with Severe Congenital Neutropenia/Acute Myeloid Leukemia, Is Required for SH2-Containing Phosphatase-1 Suppression of Stat Activation
J. Immunol., December 1, 2001; 167(11): 6447 - 6452.
[Abstract] [Full Text] [PDF]


Home page
Vet PatholHome page
B. D. Car and V. M. Eng
Special Considerations in the Evaluation of the Hematology and Hemostasis of Mutant Mice
Vet. Pathol., January 1, 2001; 38(1): 20 - 30.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
K. Nakamura, M. Kobayashi, N. Konishi, H. Kawaguchi, S.-i. Miyagawa, T. Sato, H. Toyoda, Y. Komada, S. Kojima, O. Katoh, et al.
Abnormalities of primitive myeloid progenitor cells expressing granulocyte colony-stimulating factor receptor in patients with severe congenital neutropenia
Blood, December 15, 2000; 96(13): 4366 - 4369.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
S. M. White, M. H. Alarcon, and D. J. Tweardy
Inhibition of granulocyte colony-stimulating factor-mediated myeloid maturation by low level expression of the differentiation-defective class IV granulocyte colony-stimulating factor receptor isoform
Blood, June 1, 2000; 95(11): 3335 - 3340.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
M. G. Hunter and B. R. Avalos
Granulocyte colony-stimulating factor receptor mutations in severe congenital neutropenia transforming to acute myelogenous leukemia confer resistance to apoptosis and enhance cell survival
Blood, March 15, 2000; 95(6): 2132 - 2137.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
A. C. Ward, I. Touw, and A. Yoshimura
The Jak-Stat pathway in normal and perturbed hematopoiesis
Blood, January 1, 2000; 95(1): 19 - 29.
[Full Text] [PDF]


Home page
BloodHome page
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]


Home page
NEJMHome page
B. Lowenberg, J. R. Downing, and A. Burnett
Acute Myeloid Leukemia
N. Engl. J. Med., September 30, 1999; 341(14): 1051 - 1062.
[Full Text] [PDF]


Home page
J. Exp. Med.Home page
A. C. Ward, Y. M. van Aesch, J. Gits, A. M. Schelen, J. P. de Koning, D. van Leeuwen, M. H. Freedman, and I. P. Touw
Novel Point Mutation in the Extracellular Domain of the Granulocyte Colony-stimulating Factor (G-CSF) Receptor in a Case of Severe Congenital Neutropenia Hyporesponsive to G-CSF Treatment
J. Exp. Med., August 16, 1999; 190(4): 497 - 508.
[Abstract] [Full Text] [PDF]


Home page
J. Exp. Med.Home page
M. H.A. Hermans, C. Antonissen, A. C. Ward, A. E.M. Mayen, R. E. Ploemacher, and I. P. Touw
Sustained Receptor Activation and Hyperproliferation in Response to Granulocyte Colony-stimulating Factor (G-CSF) in Mice with a Severe Congenital Neutropenia/Acute Myeloid Leukemia-derived Mutation in the G-CSF Receptor Gene
J. Exp. Med., February 15, 1999; 189(4): 683 - 692.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
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]


Home page
BloodHome page
A. C. Ward, Y. M. van Aesch, A. M. Schelen, and I. P. Touw
Defective Internalization and Sustained Activation of Truncated Granulocyte Colony-Stimulating Factor Receptor Found in Severe Congenital Neutropenia/Acute Myeloid Leukemia
Blood, January 15, 1999; 93(2): 447 - 458.
[Abstract] [Full Text] [PDF]


Home page
BloodHome page
A. C. Ward, M. H.A. Hermans, L. Smith, Y. M. van Aesch, A. M. Schelen, C. Antonissen, and I. P. Touw
Tyrosine-Dependent and -Independent Mechanisms of STAT3 Activation by the Human Granulocyte Colony-Stimulating Factor (G-CSF) Receptor Are Differentially Utilized Depending on G-CSF Concentration
Blood, January 1, 1999; 93(1): 113 - 124.
[Abstract] [Full Text] [PDF]



 click for free articles
home about blood authors subscriptions permissions advertising public access contact us
Sponsor: Genentech BioOncology and and Biogen Idec
Blood Online is supported in part by
Genentech BioOncology and Biogen Idec
  Copyright © 1998 by American Society of Hematology         Online ISSN: 1528-0020