Studies of platelets from patients with the grey platelet syndrome

Publisher Website
Google Scholar
Abstract
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 2 patients with this syndrome, the initial response of human platlets to .alpha.-thrombin does not require the presence of .alpha.-granules nor the effective release of their constituents. These platelets respond to thrombin with a normal, dose-dependent membrane potential change, and a normal secondary release of diS-C3-(5) throught 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.