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Br J Haematol. 1985 Apr;59(4):603-9.

Studies of platelets from patients with the grey platelet syndrome.


The grey platelet syndrome is a rare inherited disorder characterized by a marked decrease or absence of alpha-granules and of platelet-specific alpha-granule proteins. By utilizing platelets from two patients with this syndrome, we here demonstrate that the initial response of human platelets to alpha-thrombin does not require the presence of alpha-granules nor the effective release of their constituents. Furthermore, these platelets respond to thrombin with a normal, dose-dependent membrane potential change, and a normal secondary release of diS-C3-(5) thought to be released in parallel with beta-glucuronidase from the lysosomal granules. These results give new insight into the initial steps in the thrombin response of normal platelets.

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