Contributions of Wiskott-Aldrich syndrome family cytoskeletal regulatory adapters to immune regulation

Immunol Rev. 2009 Nov;232(1):175-94. doi: 10.1111/j.1600-065X.2009.00846.x.

Abstract

Cytoskeletal structure and dynamic rearrangement are integrally involved in coupling external stimuli to the orchestrated network of molecular interactions and cellular responses required for T-cell effector function. Members of the Wiskott-Aldrich syndrome protein (WASp) family are now widely recognized as cytoskeletal scaffolding adapters that coordinate the transmission of stimulatory signals to downstream induction of actin remodeling and cytoskeletal-dependent T-cell responses. In this review, we discuss the structural and functional properties of the WASp family members, with an emphasis on the roles of these proteins in the molecular pathways underpinning T-cell activation. The contributions of WASp family proteins and the cytoskeletal reorganization they evoke to expression of specific T-cell effector functions and the implications of such activity to normal immune responses and to the immunologic deficits manifested by Wiskott-Aldrich syndrome patients are also described.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cytoskeletal Proteins / immunology
  • Cytoskeletal Proteins / metabolism*
  • Humans
  • Immunomodulation*
  • Lymphocyte Activation
  • Protein Multimerization / immunology
  • Signal Transduction
  • T-Lymphocytes / immunology
  • T-Lymphocytes / metabolism*
  • T-Lymphocytes / pathology
  • Wiskott-Aldrich Syndrome / immunology*
  • Wiskott-Aldrich Syndrome / pathology
  • Wiskott-Aldrich Syndrome Protein Family / immunology
  • Wiskott-Aldrich Syndrome Protein Family / metabolism*

Substances

  • Cytoskeletal Proteins
  • Wiskott-Aldrich Syndrome Protein Family