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.
D001791:Blood Platelet Disorders / Q000378:metabolism / Q000503:physiopathology*; D001792:Blood Platelets / Q000503:physiopathology*; D004305:Dose-Response Relationship, Drug; D005966:Glucuronidase / Q000378:metabolism; D006801:Humans; D008564:Membrane Potentials / Q000187:drug effects; D012701:Serotonin / Q000378:metabolism; D013050:Spectrometry, Fluorescence; D013577:Syndrome; D013917:Thrombin / Q000494:pharmacology; D013997:Time Factors
D012701:Serotonin; D005966:Glucuronidase; D013917:Thrombin