Genetic deletion of mouse platelet glycoprotein Ib produces a Bernard-Soulier phenotype with increased -granule size
نویسندگان
چکیده
http://bloodjournal.hematologylibrary.org/content/104/8/2339.full.html Updated information and services can be found at: (2497 articles) Hemostasis, Thrombosis, and Vascular Biology (1086 articles) Gene Expression (790 articles) Cell Adhesion and Motility Articles on similar topics can be found in the following Blood collections http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about reproducing this article in parts or in its entirety may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#reprints Information about ordering reprints may be found online at: http://bloodjournal.hematologylibrary.org/site/subscriptions/index.xhtml Information about subscriptions and ASH membership may be found online at:
منابع مشابه
HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Genetic deletion of mouse platelet glycoprotein Ib produces a Bernard-Soulier phenotype with increased -granule size
Here we report the characterization of a mouse model of the Bernard-Soulier syndrome generated by a targeted disruption of the gene encoding the glycoprotein (GP) Ib subunit of the GP Ib-IX complex. Similar to a Bernard-Soulier model generated by disruption of the mouse GP Ib subunit, GP Ib Null mice display macrothrombocytopenia and a severe bleeding phenotype. When examined by transmission el...
متن کاملAmelioration of the macrothrombocytopenia associated with the murine Bernard-Soulier syndrome.
An absent platelet glycoprotein (GP) Ib-IX receptor results in the Bernard-Soulier syndrome and is characterized by severe bleeding and the laboratory presentation of macrothrombocytopenia. Although the macrothrombocytopenic phenotype is directly linked to an absent GP Ib-IX complex, the disrupted molecular mechanisms that produce the macrothrombocytopenia are unknown. We have utilized a mouse ...
متن کاملGeneration and rescue of a murine model of platelet dysfunction: the Bernard-Soulier syndrome.
The human Bernard-Soulier syndrome is an autosomal recessive disorder of platelet dysfunction presenting with mild thrombocytopenia, circulating "giant" platelets and a bleeding phenotype. The bleeding in patients with the Bernard-Soulier syndrome is disproportionately more severe than suggested by the reduced platelet count and is explained by a defect in primary hemostasis owing to the absenc...
متن کاملQuantitative Immunophemotyping of Platelet Surface Glycoproteins among Iranian Patients with Bernard-Soulier Syndrome
Background: Bernard-Soulier syndrome is a rare inherited bleeding disease caused by quantitative or qualitative defect of GPIb/IX/V, a platelet complex that binds the Von Willebrand factor. The expression of GPIb-IX-V complex can be evaluated by flow cytometry and confirmed by the absence of ristocetin-induced platelet aggregation in platelet-rich plasma. The main aim of the present study was t...
متن کاملDecreased thrombotic tendency in mouse models of the Bernard-Soulier syndrome.
OBJECTIVE The platelet glycoprotein (GP)Ib-V-IX complex is a receptor required for normal hemostasis deficient in the Bernard-Soulier bleeding disorder. To evaluate the consequences of GPIb-V-IX deficiency in thrombosis we generated mouse models of the disease by targeting the GPIb beta subunit. METHODS AND RESULTS Complete deletion (GPIb beta-/-) or an intracellular truncation (GPIb beta del...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره شماره
صفحات -
تاریخ انتشار 2004