|
|
 Previous Article | Table of Contents | Next Article 
Defective Actin Reorganization and Polymerization of Wiskott-Aldrich T Cells in Response to CD3-Mediated Stimulation
María Dolores Gallego,
Manuel Santamaría,
José Peña, and
Ignacio J. Molina
From the Immunology Unit, University of Granada School of Medicine, Granada, Spain; and the Department of Physiology and Immunology, "Reina Sofía" University Hospital and School of Medicine, University of Córdoba, Córdoba, Spain.
The Wiskott-Aldrich syndrome (WAS) is a severe immunodeficiency and platelet deficiency disease arising from mutation(s) in the WASP gene, which in normal cells encodes an intracellular protein able to interact with other proteins relevant to the control of cytoskeleton organization. Immunodeficiency is mainly due to T-cell progressive malfunction. Salient defects of WAS T cells are a CD3-restricted impairment in proliferative responses and cytoskeletal abnormalities, including the frequent appearance of T cells with atypical morphology. We have investigated the possibility that the CD3-restricted defect and some of the cytoskeletal defects of WAS T cells are linked. For this purpose, we immortalized by means of infection with Herpesvirus Saimiri a number of previously described allospecific WAS T-cell lines. The resulting cells preserve the surface, molecular, and functional phenotypes of their parental lines, including a negligible WASP mRNA expression as well as the CD3-restricted defect and cytoskeleton abnormalities. Results show that, in CD3-stimulated WAS T cells, the pattern of temporal changes in cell shape and F-actin distribution is substantially different from that of control cells. Furthermore, polymerization of actin, a critical step in the CD3-mediated cytoskeleton reorganization, does not occur in WAS T-cell lines in response to OKT3 stimulation. In conclusion, our data link both CD3 and cytoskeletal defects in WAS T cells, strongly suggesting that cytoskeleton abnormalities are an underlying cause for WAS immunodeficiency.
Blood, Vol. 90 No. 8 (July 15), 1997:
pp. 3089-3097
© 1997 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:
|
|
|
|
 
M. Bosticardo, F. Marangoni, A. Aiuti, A. Villa, and M. Grazia Roncarolo
Recent advances in understanding the pathophysiology of Wiskott-Aldrich syndrome
Blood,
June 18, 2009;
113(25):
6288 - 6295.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Le Bras, M. Massaad, S. Koduru, L. Kumar, M. K. Oyoshi, J. Hartwig, and R. S. Geha
WIP is critical for T cell responsiveness to IL-2
PNAS,
May 5, 2009;
106(18):
7519 - 7524.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Meyer-Bahlburg, S. Becker-Herman, S. Humblet-Baron, S. Khim, M. Weber, G. Bouma, A. J. Thrasher, F. D. Batista, and D. J. Rawlings
Wiskott-Aldrich syndrome protein deficiency in B cells results in impaired peripheral homeostasis
Blood,
November 15, 2008;
112(10):
4158 - 4169.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
I-J. Chen, H.-L. Chen, and M. Demetriou
Lateral Compartmentalization of T Cell Receptor Versus CD45 by Galectin-N-Glycan Binding and Microfilaments Coordinate Basal and Activation Signaling
J. Biol. Chem.,
November 30, 2007;
282(48):
35361 - 35372.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Badour, M. K. H. McGavin, J. Zhang, S. Freeman, C. Vieira, D. Filipp, M. Julius, G. B. Mills, and K. A. Siminovitch
Interaction of the Wiskott-Aldrich syndrome protein with sorting nexin 9 is required for CD28 endocytosis and cosignaling in T cells
PNAS,
January 30, 2007;
104(5):
1593 - 1598.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. A. de la Fuente, Y. Sasahara, M. Calamito, I. M. Anton, A. Elkhal, M. D. Gallego, K. Suresh, K. Siminovitch, H. D. Ochs, K. C. Anderson, et al.
WIP is a chaperone for Wiskott-Aldrich syndrome protein (WASP)
PNAS,
January 16, 2007;
104(3):
926 - 931.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. D. Gallego, M. A. de la Fuente, I. M. Anton, S. Snapper, R. Fuhlbrigge, and R. S. Geha
WIP and WASP play complementary roles in T cell homing and chemotaxis to SDF-1{alpha}
Int. Immunol.,
February 1, 2006;
18(2):
221 - 232.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Wada, S. H. Schurman, E. K. Garabedian, A. Yachie, and F. Candotti
Analysis of T-cell repertoire diversity in Wiskott-Aldrich syndrome
Blood,
December 1, 2005;
106(12):
3895 - 3897.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. R. Paccani, M. Boncristiano, L. Patrussi, C. Ulivieri, A. Wack, S. Valensin, T. R. Hirst, A. Amedei, G. del Prete, J. L. Telford, et al.
Defective Vav expression and impaired F-actin reorganization in a subset of patients with common variable immunodeficiency characterized by T-cell defects
Blood,
July 15, 2005;
106(2):
626 - 634.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. M. Martin-Fernandez, J. A. Cabanillas, M. Rivero-Carmena, E. Lacasa, J. Pardo, A. Anel, P. R. Ramirez-Duque, F. Merino, C. Rodriguez-Gallego, and J. R. Regueiro
Herpesvirus saimiri-transformed CD8+ T cells as a tool to study Chediak-Higashi syndrome cytolytic lymphocytes
J. Leukoc. Biol.,
May 1, 2005;
77(5):
661 - 668.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Guerra, M. Aracil, R. Conde, A. Bernad, and M. Esteban
Wiskott-Aldrich Syndrome Protein Is Needed for Vaccinia Virus Pathogenesis
J. Virol.,
February 15, 2005;
79(4):
2133 - 2140.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. Westerberg, M. Larsson, S. J. Hardy, C. Fernandez, A. J. Thrasher, and E. Severinson
Wiskott-Aldrich syndrome protein deficiency leads to reduced B-cell adhesion, migration, and homing, and a delayed humoral immune response
Blood,
February 1, 2005;
105(3):
1144 - 1152.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Burns, G. O. Cory, W. Vainchenker, and A. J. Thrasher
Mechanisms of WASp-mediated hematologic and immunologic disease
Blood,
December 1, 2004;
104(12):
3454 - 3462.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Majstoravich, J. Zhang, S. Nicholson-Dykstra, S. Linder, W. Friedrich, K. A. Siminovitch, and H. N. Higgs
Lymphocyte microvilli are dynamic, actin-dependent structures that do not require Wiskott-Aldrich syndrome protein (WASp) for their morphology
Blood,
September 1, 2004;
104(5):
1396 - 1403.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. M. Sorokina, J. J. Merlo Jr., and A. Y. Tsygankov
Molecular Mechanisms of the Effect of Herpesvirus saimiri Protein StpC on the Signaling Pathway Leading to NF-{kappa}B Activation
J. Biol. Chem.,
April 2, 2004;
279(14):
13469 - 13477.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Kettner, L. Kumar, I. M. Anton, Y. Sasahara, M. de la Fuente, V. I. Pivniouk, H. Falet, J. H. Hartwig, and R. S. Geha
WIP Regulates Signaling via the High Affinity Receptor for Immunoglobulin E in Mast Cells
J. Exp. Med.,
February 2, 2004;
199(3):
357 - 368.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Greicius, L. Westerberg, E. J. Davey, E. Buentke, A. Scheynius, J. Thyberg, and E. Severinson
Microvilli structures on B lymphocytes: inducible functional domains?
Int. Immunol.,
February 1, 2004;
16(2):
353 - 364.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Zeng, J. L. Cannon, R. T. Abraham, M. Way, D. D. Billadeau, J. Bubeck-Wardenberg, and J. K. Burkhardt
SLP-76 Coordinates Nck-Dependent Wiskott-Aldrich Syndrome Protein Recruitment with Vav-1/Cdc42-Dependent Wiskott-Aldrich Syndrome Protein Activation at the T Cell-APC Contact Site
J. Immunol.,
August 1, 2003;
171(3):
1360 - 1368.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Klein, D. Nguyen, C.-H. Liu, A. Mizoguchi, A. K. Bhan, H. Miki, T. Takenawa, F. S. Rosen, F. W. Alt, R. C. Mulligan, et al.
Gene therapy for Wiskott-Aldrich syndrome: rescue of T-cell signaling and amelioration of colitis upon transplantation of retrovirally transduced hematopoietic stem cells in mice
Blood,
March 15, 2003;
101(6):
2159 - 2166.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. O. C. Cory, R. Garg, R. Cramer, and A. J. Ridley
Phosphorylation of Tyrosine 291 Enhances the Ability of WASp to Stimulate Actin Polymerization and Filopodium Formation
J. Biol. Chem.,
November 15, 2002;
277(47):
45115 - 45121.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Yamaguchi, T. Ariga, M. Yamada, D. L. Nelson, R. Kobayashi, C. Kobayashi, Y. Noguchi, Y. Ito, K. Katamura, Y. Nagatoshi, et al.
Mixed chimera status of 12 patients with Wiskott-Aldrich syndrome (WAS) after hematopoietic stem cell transplantation: evaluation by flow cytometric analysis of intracellular WAS protein expression
Blood,
July 30, 2002;
100(4):
1208 - 1214.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Sato, N. M. Tsuji, H. Gotoh, K. Yamashita, K. Hashimoto, N. Tadotsu, H. Yamanaka, K. Sekikawa, and Y. Hashimoto
Overexpression of the Wiskott-Aldrich Syndrome Protein N-Terminal Domain in Transgenic Mice Inhibits T Cell Proliferative Responses Via TCR Signaling Without Affecting Cytoskeletal Rearrangements
J. Immunol.,
October 15, 2001;
167(8):
4701 - 4709.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. Westerberg, G. Greicius, S. B. Snapper, P. Aspenstrom, and E. Severinson
Cdc42, Rac1, and the Wiskott-Aldrich syndrome protein are involved in the cytoskeletal regulation of B lymphocytes
Blood,
August 15, 2001;
98(4):
1086 - 1094.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Rivero-Carmena, O. Porras, B. Pelaez, A. Pacheco-Castro, R. A. Gatti, and J. R. Regueiro
Membrane and transmembrane signaling in Herpesvirus saimiri-transformed human CD4+ and CD8+ T lymphocytes is ATM-independent.
Int. Immunol.,
June 1, 2000;
12(6):
927 - 935.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. Wang, H. Zhang, P. A. Solski, M. J. Hart, C. J. Der, and L. Su
Modulation of HIV-1 Replication by a Novel RhoA Effector Activity
J. Immunol.,
May 15, 2000;
164(10):
5369 - 5374.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Krause, A. S. Sechi, M. Konradt, D. Monner, F. B. Gertler, and J. Wehland
Fyn-Binding Protein (Fyb)/Slp-76-Associated Protein (Slap), Ena/Vasodilator-Stimulated Phosphoprotein (Vasp) Proteins and the Arp2/3 Complex Link T Cell Receptor (Tcr) Signaling to the Actin Cytoskeleton
J. Cell Biol.,
April 3, 2000;
149(1):
181 - 194.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. N. Savoy, D. D. Billadeau, and P. J. Leibson
Cutting Edge: WIP, a Binding Partner for Wiskott-Aldrich Syndrome Protein, Cooperates with Vav in the Regulation of T Cell Activation
J. Immunol.,
March 15, 2000;
164(6):
2866 - 2870.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. Tian, D. L. Nelson, and D. M. Stewart
Cdc42-interacting Protein 4 Mediates Binding of the Wiskott-Aldrich Syndrome Protein to Microtubules
J. Biol. Chem.,
March 10, 2000;
275(11):
7854 - 7861.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Rengan, H. D. Ochs, L. I. Sweet, M. L. Keil, W. T. Gunning, N. A. Lachant, L. A. Boxer, and G. M. Omann
Actin cytoskeletal function is spared, but apoptosis is increased, in WAS patient hematopoietic cells
Blood,
February 15, 2000;
95(4):
1283 - 1292.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. M Jones and H. B Gaspar
Immunogenetics: changing the face of immunodeficiency
J. Clin. Pathol.,
January 1, 2000;
53(1):
60 - 65.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Shcherbina, F. S. Rosen, and E. Remold-O'Donnell
WASP Levels in Platelets and Lymphocytes of Wiskott-Aldrich Syndrome Patients Correlate with Cell Dysfunction
J. Immunol.,
December 1, 1999;
163(11):
6314 - 6320.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. L. Rawlings, G. M. Crooks, D. Bockstoce, L. W. Barsky, R. Parkman, and K. I. Weinberg
Spontaneous Apoptosis in Lymphocytes From Patients With Wiskott-Aldrich Syndrome: Correlation of Accelerated Cell Death and Attenuated Bcl-2 Expression
Blood,
December 1, 1999;
94(11):
3872 - 3882.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. Zhang, A. Shehabeldin, L. A.G. da Cruz, J. Butler, A.-K. Somani, M. McGavin, I. Kozieradzki, A. O. dos Santos, A. Nagy, S. Grinstein, et al.
Antigen Receptor-Induced Activation and Cytoskeletal Rearrangement Are Impaired in Wiskott-Aldrich Syndrome Protein-Deficient Lymphocytes
J. Exp. Med.,
November 1, 1999;
190(9):
1329 - 1342.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Y. Ohashi, S. Iwata, K. Kamiguchi, and C. Morimoto
Tyrosine Phosphorylation of Crk-Associated Substrate Lymphocyte-Type Is a Critical Element in TCR- and {beta}1 Integrin-Induced T Lymphocyte Migration
J. Immunol.,
October 1, 1999;
163(7):
3727 - 3734.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Linder, D. Nelson, , M. Weiss, and M. Aepfelbacher
Wiskott-Aldrich syndrome protein regulates podosomes in primary human macrophages
PNAS,
August 17, 1999;
96(17):
9648 - 9653.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Haddad, E. Cramer, C. Riviere, P. Rameau, F. Louache, J. Guichard, D. L. Nelson, A. Fischer, W. Vainchenker, and N. Debili
The Thrombocytopenia of Wiskott Aldrich Syndrome Is Not Related to a Defect in Proplatelet Formation
Blood,
July 15, 1999;
94(2):
509 - 518.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Vaduva, N. Martinez-Quiles, I. M. Anton, N. C. Martin, R. S. Geha, A. K. Hopper, and N. Ramesh
The Human WASP-interacting Protein, WIP, Activates the Cell Polarity Pathway in Yeast
J. Biol. Chem.,
June 11, 1999;
274(24):
17103 - 17108.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. M. Stewart, L. Tian, and D. L. Nelson
Mutations That Cause the Wiskott-Aldrich Syndrome Impair the Interaction of Wiskott-Aldrich Syndrome Protein (WASP) with WASP Interacting Protein
J. Immunol.,
April 15, 1999;
162(8):
5019 - 5024.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T.S. FINCO, D. YABLONSKI, J. LIN, and A. WEISS
The Adapter Proteins LAT and SLP-76 Are Required for T-cell Activation
Cold Spring Harb Symp Quant Biol,
January 1, 1999;
64(0):
265 - 274.
[Abstract]
[PDF]
|
|
|
|
|
|
|
Y. Wu, D. Dowbenko, and L. A. Lasky
PSTPIP 2, a Second Tyrosine Phosphorylated, Cytoskeletal-associated Protein That Binds a PEST-type Protein-tyrosine Phosphatase
J. Biol. Chem.,
November 13, 1998;
273(46):
30487 - 30496.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Pacheco-Castro, D. Alvarez-Zapata, P. Serrano-Torres, and J. R. Regueiro
Signaling Through a CD3{gamma}-Deficient TCR/CD3 Complex in Immortalized Mature CD4+ and CD8+ T Lymphocytes
J. Immunol.,
September 15, 1998;
161(6):
3152 - 3160.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Oda, H. D. Ochs, B. J. Druker, K. Ozaki, C. Watanabe, M. Handa, Y. Miyakawa, and Y. Ikeda
Collagen Induces Tyrosine Phosphorylation of Wiskott-Aldrich Syndrome Protein in Human Platelets
Blood,
September 15, 1998;
92(6):
1852 - 1858.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. E. Bear, J. F. Rawls, and C. L. Saxe III
SCAR, a WASP-related Protein, Isolated as a Suppressor of Receptor Defects in Late Dictyostelium Development
J. Cell Biol.,
September 7, 1998;
142(5):
1325 - 1335.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
