The expression of Wiskott-Aldrich syndrome protein (WASP) is dependent on WASP-interacting protein (WIP)

Int Immunol. 2007 Feb;19(2):185-92. doi: 10.1093/intimm/dxl135. Epub 2007 Jan 6.

Abstract

The Wiskott-Aldrich syndrome protein (WASP) is a key molecule for transduction of extracellular signals that induce a variety of critical biological events involving actin cytoskeleton rearrangement. Among the cellular partners of WASP, the Wiskott-Aldrich syndrome protein-interacting protein (WIP) has been speculated to play a critical role in the pathophysiology of Wiskott-Aldrich syndrome since WASP mutation hot spots map to the WIP-binding region. The notion that WIP promotes WASP function, however, conflicts with evidence that WIP inhibits WASP-mediated actin polymerization and IL-2 production and suggests a complex regulation of WASP function by WIP. Here we show that WASP gene transfer results in high WASP expression only when WIP is concomitantly expressed in K562 cells. Furthermore, WIP-knockdown experiments demonstrated that T cells with reduced WIP expression show a concordant reduction of WASP levels. Mapping studies using WIP mutants showed that the minimal WIP region able to rescue WASP expression in WIP-knockdown cells was the WASP-binding domain. However, expression of such a minimal domain of WIP failed to rescue WASP-dependent, nuclear factor of activated T-cells-mediated IL-2 transcriptional activity. These results demonstrate that expression of WIP is necessary for functional WASP expression in human cells and provide a new paradigm for understanding the function of these two molecules.

Publication types

  • Research Support, N.I.H., Intramural

MeSH terms

  • Blotting, Western
  • Carrier Proteins / metabolism*
  • Cell Line
  • Cytoskeletal Proteins
  • Flow Cytometry
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • RNA Interference
  • Reverse Transcriptase Polymerase Chain Reaction
  • Transfection
  • Wiskott-Aldrich Syndrome Protein / biosynthesis*

Substances

  • Carrier Proteins
  • Cytoskeletal Proteins
  • Intracellular Signaling Peptides and Proteins
  • WIPF1 protein, human
  • Wiskott-Aldrich Syndrome Protein