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Platelets ; 20(3): 216-24, 2009 May.
Artículo en Inglés | MEDLINE | ID: mdl-19437340

RESUMEN

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.


Asunto(s)
Plaquetas/metabolismo , Factor de Transcripción GATA1/metabolismo , Enfermedades Genéticas Ligadas al Cromosoma X/sangre , Talina/sangre , Plaquetas/ultraestructura , Membrana Celular/metabolismo , Membrana Celular/ultraestructura , Factor de Transcripción GATA1/genética , Humanos , Técnicas In Vitro , Mutación
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