GATA-1, G208S macrothrombocytes are deficient in talin: immunofluorescence studies.
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.
White
James G
JG
Department of Laboratory Medicine, Pathology and Pediatrics, University of Minnesota School of Medicine, Minneapolis, MN, USA. White003@umn.edu
Burris
Steven M
SM
Crowe
Brian R
BR
D001792:Blood Platelets / Q000378:metabolism* / Q000648:ultrastructure; D002462:Cell Membrane / Q000378:metabolism / Q000648:ultrastructure; D050982:GATA1 Transcription Factor / Q000235:genetics / Q000378:metabolism*; D040181:Genetic Diseases, X-Linked / Q000097:blood*; D006801:Humans; D066298:In Vitro Techniques; D009154:Mutation; D016608:Talin / Q000097:blood*
D050982:GATA1 Transcription Factor; C494688:GATA1 protein, human; D016608:Talin