hematopoietic system
• mild thrombocytopenia
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• secretion of alpha and dense granules after PMA stimulation is impaired
• 2 minutes after PMA stimulation no fusion of alpha and dense granules is seen unlike in wild-type platelets
• despite similar levels of granule fusion by 5 minutes after stimulation, granule fusion with cell surface membranes is still impaired and the contents of the granules are not secreted into the extracellular space
• however, granule secretion after thrombin stimulation is normal
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• mild impairment in aggregation of washed platelets in response to low level thrombin receptor agonist stimulation
• aggregation defect of washed platelets is more severe when stimulated with PKC activators like phorbol esters (PMA)
• addition of fibrinogen to washed platelets corrects the aggregation defect
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homeostasis/metabolism
• secretion of alpha and dense granules after PMA stimulation is impaired
• 2 minutes after PMA stimulation no fusion of alpha and dense granules is seen unlike in wild-type platelets
• despite similar levels of granule fusion by 5 minutes after stimulation, granule fusion with cell surface membranes is still impaired and the contents of the granules are not secreted into the extracellular space
• however, granule secretion after thrombin stimulation is normal
|
• mild impairment in aggregation of washed platelets in response to low level thrombin receptor agonist stimulation
• aggregation defect of washed platelets is more severe when stimulated with PKC activators like phorbol esters (PMA)
• addition of fibrinogen to washed platelets corrects the aggregation defect
|