DOI: 10.4049/jimmunol.161.2.1026. Monocytes from Wiskott-Aldrich Patients Display Reduced Chemotaxis and Lack of Cell Polarization in Response to Monocyte Chemoattractant Protein-1 and Formyl-Methionyl-Leucyl-Phenylalanine

Monocytes from Wiskott-Aldrich Patients Display Reduced Chemotaxis and Lack of Cell Polarization in Response to Monocyte Chemoattractant Protein-1 and Formyl-Methionyl-Leucyl-Phenylalanine

10.4049/jimmunol.161.2.1026

Crossref journal-article

Oxford University Press (OUP)

The Journal of Immunology (286)

Abstract

AbstractWiskott-Aldrich syndrome (WAS) is an X-linked disorder characterized by trombocytopenia, eczema, and progressive decline of the immune function. In addition, lymphocytes and platelets from WAS patients have morphologic abnormalities. Since chemokines may induce morphologic changes and migration of leukocytes, we investigated the monocyte response to chemoattractants in cells from WAS patients with an identified mutation in the WAS protein gene. Here, we report that monocytes derived from four patients with molecularly defined typical WAS have a severely impaired migration in response to FMLP and to the chemokines monocyte chemoattractant protein-1 (MCP-1) and macrophage inflammatory protein-1α compared with normal donors. Conversely, neither MCP-1 binding to monocytes nor induction of the respiratory burst by MCP-1 and FMLP is significantly different between WAS patients and normal donors. Within a few minutes of stimulation, monocytes respond to chemokines with increased expression of adhesion molecules and with morphologic changes such as cell polarization. Although up-regulation of CD11b/CD18 expression following stimulation with FMLP or MCP-1 is preserved in WAS patients, cell polarization is dramatically decreased. Staining of F-actin by FITC-phalloidin in monocytes stimulated with chemoattractants shows F-actin to have a rounded shape in WAS patients, as opposed to the polymorphic distribution of F-actin in the polarized monocytes from healthy donors. These results suggest that WAS protein is involved in the monocyte response to the chemokines MCP-1 and macrophage inflammatory protein-1α.

Bibliography

Badolato, R., Sozzani, S., Malacarne, F., Bresciani, S., Fiorini, M., Borsatti, A., Albertini, A., Mantovani, A., Ugazio, A. G., & Notarangelo, L. D. (1998). Monocytes from Wiskott-Aldrich Patients Display Reduced Chemotaxis and Lack of Cell Polarization in Response to Monocyte Chemoattractant Protein-1 and Formyl-Methionyl-Leucyl-Phenylalanine. The Journal of Immunology, 161(2), 1026â1033.

Authors 10

Raffaele Badolato (first)
Silvano Sozzani (additional)
Fabio Malacarne (additional)
Susanna Bresciani (additional)
Maurilia Fiorini (additional)
Alessandro Borsatti (additional)
Alberto Albertini (additional)
Alberto Mantovani (additional)
Alberto G. Ugazio (additional)
Luigi D. Notarangelo (additional)

References 40 Referenced 121

Dates

Type	When
Created	2 years, 7 months ago (Dec. 31, 2022, 9:20 a.m.)
Deposited	7 months, 3 weeks ago (Jan. 2, 2025, 11:56 a.m.)
Indexed	1 month, 3 weeks ago (July 2, 2025, 1:56 p.m.)
Issued	27 years, 1 month ago (July 15, 1998)
Published	27 years, 1 month ago (July 15, 1998)
Published Print	27 years, 1 month ago (July 15, 1998)

Funders 0

None

BibTeX

@article{Badolato_1998, title={Monocytes from Wiskott-Aldrich Patients Display Reduced Chemotaxis and Lack of Cell Polarization in Response to Monocyte Chemoattractant Protein-1 and Formyl-Methionyl-Leucyl-Phenylalanine}, volume={161}, ISSN={1550-6606}, url={http://dx.doi.org/10.4049/jimmunol.161.2.1026}, DOI={10.4049/jimmunol.161.2.1026}, number={2}, journal={The Journal of Immunology}, publisher={Oxford University Press (OUP)}, author={Badolato, Raffaele and Sozzani, Silvano and Malacarne, Fabio and Bresciani, Susanna and Fiorini, Maurilia and Borsatti, Alessandro and Albertini, Alberto and Mantovani, Alberto and Ugazio, Alberto G. and Notarangelo, Luigi D.}, year={1998}, month=jul, pages={1026–1033} }

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