WASP deficiency leads to global defects of directed leukocyte migration in vitro and in vivo

J Leukoc Biol. 2005 Jun;77(6):993-8. doi: 10.1189/jlb.0804444. Epub 2005 Mar 17.

Abstract

Intact cellular migration is critically important for the induction and regulation of the immune response. The Wiskott-Aldrich syndrome protein (WASP) regulates surface receptor signaling to the actin cytoskeleton in hematopoietic cells and thus plays a pivotal role in cellular locomotion. WASP deficiency causes the Wiskott-Aldrich syndrome (WAS), characterized by immunodeficiency, thrombocytopenia, and eczema. Cell migration defects may contribute to the pathophysiology of WAS. In this study, we used a variety of in vitro and in vivo assays to comprehensively analyze migration properties of lymphocytes, dendritic cells (DC), and neutrophils from WASP-deficient mice. We provide evidence that WASP-deficient lymphocytes show a marked reduction in tethering in an in vitro flow chamber assay as well as decreased migration of T cells in response to the CC chemokine ligand 19 (CCL19). In vivo, compared with wild-type lymphocytes, WASP-deficient lymphocytes showed significantly impaired homing to Peyer's patches upon adoptive transfer into recipient mice. In addition, bone marrow-derived DC migrated less efficiently in response to CCL19. In vivo studies showed decreased migration of DC from skin to draining lymph nodes in WASP-deficient animals. Finally, we also document decreased neutrophil migration in vitro and in vivo. In summary, our studies suggest that WASP plays an important role in the locomotion of lymphocytes, DC, and granulocytes in vitro and in vivo and thus, reveal a crucial role of WASP in physiological trafficking of various hematopoietic cell lineages. These results further delineate immunological abnormalities in WASP-deficient mice, which will be useful to assess preclinical gene therapy studies.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Cell Movement / physiology*
  • Chemokine CCL19
  • Chemokines, CC / pharmacology
  • Dendritic Cells / drug effects
  • Dendritic Cells / physiology
  • Leukocytes / drug effects
  • Leukocytes / physiology*
  • Mice
  • Mice, Mutant Strains
  • Mutation
  • Neutrophils / drug effects
  • Neutrophils / physiology
  • Proteins / genetics
  • Proteins / physiology*
  • T-Lymphocytes / drug effects
  • T-Lymphocytes / physiology
  • Wiskott-Aldrich Syndrome Protein

Substances

  • Ccl19 protein, mouse
  • Chemokine CCL19
  • Chemokines, CC
  • Proteins
  • Was protein, mouse
  • Wiskott-Aldrich Syndrome Protein