GATA-1, G208S macrothrombocytes are deficient in talin: immunofluorescence studies

Platelets. 2009 May;20(3):216-24. doi: 10.1080/09537100902818344.

Abstract

Previous investigations from our laboratory identified the ultrastructural pathology and cytochemistry of macrothrombocytes (MTC) from patients with the X-linked, G208S varient of the GATA-1 mutation.A subsequent biochemical study of the MTC cytoskeletal proteins using polyacrylamide gel electrophoresis and western blot analysis revealed the MTC were deficient in the high-molecular weight, actin binding protein, talin. The present study has used immunofluorescent techniques to further characterize the talin deficiency. Results confirm that the GATA-1, G208S MTC are deficient in talin, and what little is present relocates to the undersurface of the plasma membrane following activations where it associates with adhesion plaques.

MeSH terms

  • Blood Platelets / metabolism*
  • Blood Platelets / ultrastructure
  • Cell Membrane / metabolism
  • Cell Membrane / ultrastructure
  • GATA1 Transcription Factor / genetics
  • GATA1 Transcription Factor / metabolism*
  • Genetic Diseases, X-Linked / blood*
  • Humans
  • In Vitro Techniques
  • Mutation
  • Talin / blood*

Substances

  • GATA1 Transcription Factor
  • GATA1 protein, human
  • Talin