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Blood, 1 February 2005, Vol. 105, No. 3, pp. 1144-1152.
Prepublished online as a Blood First Edition Paper on September 21, 2004; DOI 10.1182/blood-2004-03-1003.
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IMMUNOBIOLOGY
Wiskott-Aldrich syndrome protein deficiency leads to reduced B-cell adhesion, migration, and homing, and a delayed humoral immune response
Lisa Westerberg,
Malin Larsson,
Samantha J. Hardy,
Carmen Fernández,
Adrian J. Thrasher, and
Eva Severinson
From the Department of Cell and Molecular Biology, Medical Nobel Institute, Karolinska Institutet, Stockholm, Sweden; the Institute of Child Health, University College London, London, United Kingdom; and the Department of Immunology, The Wenner-Gren Institute, Stockholm University, Stockholm, Sweden.
The Wiskott-Aldrich syndrome protein (WASp) is mutated in the severe immunodeficiency disease Wiskott-Aldrich syndrome (WAS). The function of B cells and the physiologic alterations in WAS remain unclear. We show that B cells from WAS patients exhibited decreased motility and had reduced capacity to migrate, adhere homotypically, and form long protrusions after in vitro culture. WASp-deficient murine B cells also migrated less well to chemokines. Upon antigen challenge, WASp-deficient mice mounted a reduced and delayed humoral immune response to both T-cell–dependent and –independent antigens. This was at least in part due to deficient migration and homing of B cells. In addition, the germinal center reaction was reduced in WASp-deficient mice. Thus, WASp is crucial for optimal B-cell responses and plays a pivotal role in the primary humoral immune response.
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