title: Analysis of platelet cytoskeleton assembly during platelet activation in Hermansky-Pudlak syndrome and thrombasthenia.
abstract: Time course change of the platelet cytoskeletal protein component in the Triton X-100 insoluble fraction after stimulation was analyzed in Hermansky-Pudlak syndrome and thrombasthenia. In Hermansky-Pudlak syndrome (HPS), a 31 kDa protein, myosin, actin, and a 100 kDa protein assembled as in the normal platelets at the shape change and release reaction phases after ADP or collagen stimulation, suggesting that, the deficient dense granule content do not lead to an abnormal platelet cytoskeletal protein assembly. In thrombasthenia (Type I), myosin increased at the shape change and release reaction phases as it does in normal platelets, but actin and the 100 kDa protein increased only at the initial activation phase, and then subsequently decreased to the level of the resting phase. The actin-binding protein (ABP) and the 31 kDa protein increased a little following stimulation. Similar cytoskeletal protein change after stimulation were found in normal platelets which were prevented from the aggregation process by chelating the external Ca2+ or by using synthetic decapeptide of fibrinogen gamma-chain of carboxyl terminus. The decreased platelet cytoskeletal protein assembly in thrombasthenia or in platelets stimulated without aggregation, was derived from a loss of the platelet aggregation process due to the defect of GP IIb-IIIa complex or an interaction failure between GP IIb-IIIa complex and fibrinogen. The interaction between platelets and either fibrinogen or fibrin can induce a more stable platelet cytoskeletal protein assembly, however, agonistic stimulation without these interactions cannot do it directly.
authors.affiliation: Department of Internal Medicine, Hiroshima University, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Fujimura
authors.forename: T
authors.initials: T
authors.lastname: Fujimoto
authors.forename: M
authors.initials: M
authors.lastname: Takemoto
authors.forename: K
authors.initials: K
authors.lastname: Oda
authors.forename: T
authors.initials: T
authors.lastname: Shimomura
authors.forename: S
authors.initials: S
authors.lastname: Maehama
authors.forename: A
authors.initials: A
authors.lastname: Kuramoto
mesh_terms: D000199:Actins / Q000097:blood; D000244:Adenosine Diphosphate / Q000494:pharmacology; D000328:Adult; D001791:Blood Platelet Disorders / Q000097:blood*; D001792:Blood Platelets / Q000473:pathology*; D002648:Child; D002850:Chromatography, Gel; D003094:Collagen / Q000494:pharmacology; D003599:Cytoskeleton / Q000473:pathology*; D003902:Detergents; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D006474:Hemorrhagic Disorders / Q000097:blood*; D006801:Humans; D008297:Male; D017830:Octoxynol; D015539:Platelet Activation; D010974:Platelet Aggregation / Q000187:drug effects; D011092:Polyethylene Glycols; D013577:Syndrome; D013915:Thrombasthenia / Q000097:blood*
chemical_list: D000199:Actins; D003902:Detergents; D011092:Polyethylene Glycols; D000244:Adenosine Diphosphate; D017830:Octoxynol; D003094:Collagen