Evidence that the Wiskott-Aldrich syndrome protein may be involved in lymphoid cell signaling pathways

J Immunol. 1996 Nov 1;157(9):3791-5.

Abstract

Wiskott-Aldrich syndrome is an X-linked combined immunodeficiency affecting cells of several different hemopoietic lineages. The Wiskott-Aldrich syndrome protein (WASP), which has no homology with any other known protein families, is rich in proline motifs known to contribute to Src homology 3 binding sites. However, its function has not been determined. The Tec family of cytoplasmic tyrosine kinases, which include Btk (the X-linked agammaglobulinemia gene), Itk, and Tec, is thought to be involved in lymphoid cell signaling pathways. In this work, we show binding of WASP to the Src homology 3 domains of Btk, Itk, Tec, Grb2, and phospholipase C-gamma, which suggests a function for WASP in lymphoid cell signaling.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • B-Lymphocytes / metabolism*
  • Binding Sites
  • Burkitt Lymphoma / pathology
  • Cell Differentiation
  • Cell Line, Transformed
  • Gene Expression
  • Humans
  • Molecular Sequence Data
  • Peptide Fragments / metabolism
  • Proline / chemistry
  • Protein Binding
  • Proteins / chemistry
  • Proteins / physiology*
  • Recombinant Fusion Proteins / metabolism
  • Signal Transduction / physiology*
  • Tumor Cells, Cultured
  • Wiskott-Aldrich Syndrome / genetics*
  • Wiskott-Aldrich Syndrome / immunology
  • Wiskott-Aldrich Syndrome / pathology
  • Wiskott-Aldrich Syndrome Protein
  • src Homology Domains / physiology*
  • src-Family Kinases / chemistry
  • src-Family Kinases / metabolism*

Substances

  • Peptide Fragments
  • Proteins
  • Recombinant Fusion Proteins
  • WAS protein, human
  • Wiskott-Aldrich Syndrome Protein
  • Proline
  • src-Family Kinases