Antigen Receptor-induced Activation and Cytoskeletal Rearrangement Are Impaired in Wiskott-Aldrich Syndrome Protein-deficient Lymphocytes

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© The Rockefeller University Press, 0022-1007/1999/11/1329/ $5.00
The Journal of Experimental Medicine, Volume 190, Number 9, November 1, 1999 1329-1342

Original Article

Antigen Receptor–induced Activation and Cytoskeletal Rearrangement Are Impaired in Wiskott-Aldrich Syndrome Protein–deficient Lymphocytes

Jinyi Zhang^a,b,c,h, Amro Shehabeldin^a,b,c,h, Luis A.G. da Cruz^a,b,c,h, Jeffrey Butler^d,g, Ally-Khan Somani^a,b,c,h, Mary McGavin^a,b,c,h, Ivona Kozieradzki^b,e,f, Antonio O. dos Santos^b,e,f, Andras Nagy^c,h, Sergio Grinstein^d,g, Josef M. Penninger^b,e,f, and Katherine A. Siminovitch^a,b,c,h
^a Department of Medicine, University of Toronto, Ontario, Canada M5G 1X5
^b Department of Immunology, University of Toronto, Ontario, Canada M5G 1X5
^c Department of Medical Genetics and Microbiology, University of Toronto, Ontario, Canada M5G 1X5
^d Department of Biochemistry, University of Toronto, Ontario, Canada M5G 1X5
^e Department of Medical Biophysics, University of Toronto, Ontario, Canada M5G 1X5
^f Amgen Institute, Ontario Cancer Institute, Toronto, Ontario, Canada M5G 2C1
^g Division of Cell Biology, Research Institute, Hospital for Sick Children
^h Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario, Canada M5G 1X5

Correspondence to: Katherine A. Siminovitch, Mount Sinai Hospital, Rm. 656A, 600 University Ave., Toronto, Ontario, Canada M5G 1X5. Tel:416-586-4692 Fax:416-586-8558 E-mail:ksimin{at} mshri.on.ca.

The Wiskott-Aldrich syndrome protein (WASp) has been implicatedin modulation of lymphocyte activation and cytoskeletal reorganization.To address the mechanisms whereby WASp subserves such functions,we have examined WASp roles in lymphocyte development and activationusing mice carrying a WAS null allele (WAS^-^/^-). Enumerationof hemopoietic cells in these animals revealed total numbersof thymocytes, peripheral B and T lymphocytes, and plateletsto be significantly diminished relative to wild-type mice. Inthe thymus, this abnormality was associated with impaired progressionfrom the CD44^-CD25⁺ to the CD44^-CD25^- stage of differentiation.WASp-deficient thymocytes and T cells also exhibited impairedproliferation and interleukin (IL)-2 production in responseto T cell antigen receptor (TCR) stimulation, but proliferatednormally in response to phorbol ester/ionomycin. This defectin TCR signaling was associated with a reduction in TCR-evokedupregulation of the early activation marker CD69 and in TCR-triggeredapoptosis. While induction of TCR- ${zeta}$ , ZAP70, and total proteintyrosine phosphorylation as well as mitogen-activated proteinkinase (MAPK) and stress-activated protein/c-Jun NH₂-terminalkinase (SAPK/JNK) activation appeared normal in TCR-stimulatedWAS^-^/^- cells, TCR-evoked increases in intracellular calciumconcentration were decreased in WASp-deficient relative to wild-typecells. WAS^-^/^- lymphocytes also manifested a marked reductionin actin polymerization and both antigen receptor capping andendocytosis after TCR stimulation, whereas WAS^-^/^- neutrophilsexhibited reduced phagocytic activity. Together, these resultsprovide evidence of roles for WASp in driving lymphocyte development,as well as in the translation of antigen receptor stimulationto proliferative or apoptotic responses, cytokine production,and cytoskeletal rearrangement. The data also reveal a rolefor WASp in modulating endocytosis and phagocytosis and, accordingly,suggest that the immune deficit conferred by WASp deficiencyreflects the disruption of a broad range of cellular behaviors.

Key Words: immunodeficiency, Wiskott-Aldrich syndrome protein, antigenreceptor signaling, cytoskeletal rearrangement, lymphocyte activation

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