title: Defective contact activation of platelets from dogs with basset hound hereditary thrombopathy.
abstract: Basset Hound Hereditary Thrombopathy (BHT) is an autosomally inherited disorder of platelet function characterized by a thrombasthenia-like defect in aggregation but normal clot retraction. Glycoprotein IIb-IIIa (GP IIb-IIIa) is detectable in BHT platelets but may be functionally defective. In order to further characterize this potential model for human Glanzmann's thrombasthenia, contact reactivity of BHT platelets was studied by whole mount electron microscopy. Gel filtered BHT platelets, after 30 minutes of contact activation, attached poorly to a formvar substrate. There was an 8 fold difference in the number of adherent BHT platelets and normal platelets. In addition, contact induced shape change was inhibited when compared to control dogs. Almost 95% of control platelets reached fully dendritic or spread forms after 30 minutes of contact; in contrast only 63.7% of BHT platelets reached this degree of activation. The addition of 8.2 uM ADP to BHT platelets induced nearly a 4 fold increase in the number of spread forms and a 5 fold increase in the number of adherent BHT platelets, but did not cause aggregate formation. Both the defect in adhesion and shape change and the ability of ADP to stimulate both adhesion and contact-induced shape change in BHT platelets are similar to recent observations in our laboratory in patient's with type II Glanzmann's thrombasthenia.
mesh_terms: D000244:Adenosine Diphosphate / Q000494:pharmacology; D000818:Animals; D001778:Blood Coagulation Disorders / Q000235:genetics / Q000662:veterinary*; D001792:Blood Platelets / Q000187:drug effects / Q000502:physiology; D004283:Dog Diseases / Q000235:genetics*; D004285:Dogs; D010973:Platelet Adhesiveness / Q000187:drug effects; D010979:Platelet Function Tests
chemical_list: D000244:Adenosine Diphosphate