Structure of the N-WASP EVH1 domain-WIP complex: insight into the molecular basis of Wiskott-Aldrich Syndrome

Cell. 2002 Nov 15;111(4):565-76. doi: 10.1016/s0092-8674(02)01076-0.

Abstract

Missense mutants that cause the immune disorder Wiskott-Aldrich Syndrome (WAS) map primarily to the Enabled/VASP homology 1 (EVH1) domain of the actin regulatory protein WASP. This domain has been implicated in both peptide and phospholipid binding. We show here that the N-WASP EVH1 domain does not bind phosphatidyl inositol-(4,5)-bisphosphate, as previously reported, but does specifically bind a 25 residue motif from the WASP Interacting Protein (WIP). The NMR structure of the complex reveals a novel recognition mechanism-the WIP ligand, which is far longer than canonical EVH1 ligands, wraps around the domain, contacting a narrow but extended surface. This recognition mechanism provides a basis for understanding the effects of mutations that cause WAS.

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Carrier Proteins / chemistry*
  • Carrier Proteins / metabolism
  • Cytoskeletal Proteins
  • Ligands
  • Models, Molecular
  • Molecular Sequence Data
  • Nerve Tissue Proteins / chemistry*
  • Nerve Tissue Proteins / metabolism
  • Peptides / metabolism
  • Phosphatidylinositol 4,5-Diphosphate / chemistry*
  • Phosphatidylinositol 4,5-Diphosphate / metabolism
  • Proline
  • Protein Structure, Tertiary
  • Rats
  • Wiskott-Aldrich Syndrome Protein, Neuronal
  • Wiskott-Aldrich Syndrome*

Substances

  • Carrier Proteins
  • Cytoskeletal Proteins
  • Ligands
  • Nerve Tissue Proteins
  • Peptides
  • Phosphatidylinositol 4,5-Diphosphate
  • Wasl protein, rat
  • Wipf1 protein, rat
  • Wiskott-Aldrich Syndrome Protein, Neuronal
  • Proline