Possible thrombosthenin defect in Glanzmann's thrombasthenia.
نویسندگان
چکیده
منابع مشابه
Identification of an abnormal gene for the GPIIIa subunit of the platelet fibrinogen receptor resulting in Glanzmann's thrombasthenia.
The platelet fibrinogen receptor, which is composed of glycoproteins IIb (GPIIb) and IIIa (GPIIIa), belongs to a large family of receptors that participate in a multitude of biologically important adhesive interactions. Platelets from most patients with the autosomal recessive bleeding disorder, Glanzmann's thrombasthenia, are deficient in GPIIb and GPIIIa. We have used cDNA probes to analyze t...
متن کاملDeletion of the platelet-specific alloantigen PlA1 from platelets in Glanzmann's thrombasthenia.
Expression of a Platelet-specific alloantigen (Pl(A1)) was studied in five unrelated patients with Glanzmann's thrombasthenia using immunologic techniques based on release of (51)Cr from tagged platelets by Pl(A1)-specific antibody. Less than 1% of the normal quantity of Pl(A1) could be detected on platelets of patients 1, 2, and 3; platelets from patients 4 and 5 contained 22 and 12% of normal...
متن کاملProtein A Sepharose immunoadsorption can restore the efficacy of platelet concentrates in patients with Glanzmann's thrombasthenia and anti-glycoprotein IIb-IIIa antibodies.
Type I Glanzmann's thrombasthenia is a rare congenital platelet function disorder, characterized by undetectable platelet membrane glycoprotein IIb-IIIa (GPIIb-IIIa). Severe bleeding is controlled by transfusion of normal platelets, leading in some cases to the occurrence of anti-GPIIb-IIIa isoantibodies, which induces a loss of transfused platelet efficacy. We used immunoadsorption on protein ...
متن کاملDental Considerations in the Management of Glanzmann’s Thrombasthenia
Glanzmann's thrombasthenia, is one of the rarest congenital, genetically inherited platelet disorder. It has an incidence of about 1:1,000,000, but is more common in populations with increased consanguinity. Glanzmann's thrombasthenia is characterized by deficiency or dysfunction of glycoprotein (GP) lib and Ilia, which are the receptors of fibrinogen. Both sexes are equally affected. Typical m...
متن کاملThe molecular genetic basis of Glanzmann's thrombasthenia in a gypsy population in France: identification of a new mutation on the alpha IIb gene.
Glanzmann's thrombasthenia is a rare inherited bleeding disorder caused by a qualitative or quantitative defect of platelet alpha IIb beta 3. We describe here a new mutation that is the molecular genetic basis of Glanzmann's thrombasthenia in two gypsy families. Our investigation was focused on the alpha IIb gene as a result of biochemical and immunologic analysis of patients' platelets showing...
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ورودعنوان ژورنال:
- Blood
دوره 39 3 شماره
صفحات -
تاریخ انتشار 1972