A novel interaction between FlnA and Syk regulates platelet ITAM-mediated receptor signaling and function

J Exp Med. 2010 Aug 30;207(9):1967-79. doi: 10.1084/jem.20100222. Epub 2010 Aug 16.

Abstract

Filamin A (FlnA) cross-links actin filaments and connects the Von Willebrand factor receptor GPIb-IX-V to the underlying cytoskeleton in platelets. Because FlnA deficiency is embryonic lethal, mice lacking FlnA in platelets were generated by breeding FlnA(loxP/loxP) females with GATA1-Cre males. FlnA(loxP/y) GATA1-Cre males have a macrothrombocytopenia and increased tail bleeding times. FlnA-null platelets have decreased expression and altered surface distribution of GPIbalpha because they lack the normal cytoskeletal linkage of GPIbalpha to underlying actin filaments. This results in approximately 70% less platelet coverage on collagen-coated surfaces at shear rates of 1,500/s, compared with wild-type platelets. Unexpectedly, however, immunoreceptor tyrosine-based activation motif (ITAM)- and ITAM-like-mediated signals are severely compromised in FlnA-null platelets. FlnA-null platelets fail to spread and have decreased alpha-granule secretion, integrin alphaIIbbeta3 activation, and protein tyrosine phosphorylation, particularly that of the protein tyrosine kinase Syk and phospholipase C-gamma2, in response to stimulation through the collagen receptor GPVI and the C-type lectin-like receptor 2. This signaling defect was traced to the loss of a novel FlnA-Syk interaction, as Syk binds to FlnA at immunoglobulin-like repeat 5. Our findings reveal that the interaction between FlnA and Syk regulates ITAM- and ITAM-like-containing receptor signaling and platelet function.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Motifs
  • Animals
  • Blood Platelets / cytology
  • Blood Platelets / metabolism*
  • Cell Adhesion
  • Female
  • Filamins
  • Intracellular Signaling Peptides and Proteins / metabolism*
  • Male
  • Mice
  • Mice, Knockout
  • Nerve Tissue Proteins / deficiency
  • Nerve Tissue Proteins / metabolism*
  • Phosphotyrosine / metabolism
  • Protein Binding
  • Protein-Tyrosine Kinases / metabolism*
  • Signal Transduction*
  • Syk Kinase
  • Thrombocytosis / genetics
  • Thrombocytosis / metabolism

Substances

  • Filamins
  • FlnA protein, mouse
  • Intracellular Signaling Peptides and Proteins
  • Nerve Tissue Proteins
  • Phosphotyrosine
  • Protein-Tyrosine Kinases
  • Syk Kinase
  • Syk protein, mouse