Studies of platelets from patients with the grey platelet syndrome.

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 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.

• Authors: S M Greenberg-Sepersky, E R Simons, J G White
• Journal: British journal of haematology (Br J Haematol) Vol. 59 Issue 4 Pg. 603-9 (Apr 1985) ISSN: 0007-1048 [Print] England
• PMID: 3986134 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Serotonin
• Glucuronidase
• Thrombin

Topics

• Blood Platelet Disorders (metabolism, physiopathology)
• Blood Platelets (physiopathology)
• Dose-Response Relationship, Drug
• Glucuronidase (metabolism)
• Humans
• Membrane Potentials (drug effects)
• Serotonin (metabolism)
• Spectrometry, Fluorescence
• Syndrome
• Thrombin (pharmacology)
• Time Factors