Thrombasthenia

GT Glanzmann Thrombasthenia GTP Guanosine triphosphate GTP-y-S Guanarine 5 0-(3-thiotriphosphate)... 

P3 pS integrin Osteopetrosis developing in adult animals Glanzmann s thrombasthenia... 

The von Willebrandt s factor plays a key role in thrombogenesis. Lack of this factor causes thrombasthenia, a pathologically decreased platelet aggregation. Relative deficiency of the von Wille-Liillmann, Color Atlas of Pharmacology... 

Glanzmann s thrombasthenia Autosomal recessive bleeding disorder absence, reduction, or dysfunction of GPIIb/IIIa complex... 

Defects of platelet aggregation—these causing (a) Glanzmann s thrombasthenia and (b) congenital anbrogenemia. 

Hodivala-Dilke KM, McHugh KP, Tsakiris DA et al. (1999) 3 3-integrin-deficient mice are a model for Glanzmann thrombasthenia showing placental defects and reduced survival. J Clin Invest 103 229-238... 

Bleeding disorder similar to Glanzmann thrombasthenia in man platelets failed to bind fibrinogen, to aggregate and to retract a fibrinogen clot a-granules do not contain fibrinogen (Tronik-Le Roux et al. 2000). 

Viable, fertile, increased fetal mortality features of Glanzmann thrombasthenia in man, e.g. defective platelet aggregation, clot retraction, spontaneous bleeding, prolonged bleeding times dysfunctional osteoclasts, development of osteosclerosis with age (Hodivala-Dilke et al. 1999 McHugh et al. 2000). 

Qemetson KJ, Clemetson JM. Molecular abnormalities in 01an2mann s thrombasthenia, Bemard-Soulier syndrome, and platelet-type von Wiliebrand s disease. CurrOpin Hematol 1994 1 388-93. 

Caen JP, Castaldi PA, Leclerc JC, et al. Congenital bleeding disorders with long bleeding times and normal platelet count Glanamann s thrombasthenia. Report of 15 patients. Am J Med 1966 41 4-26. 

Hardisty RM, Dormandy KM, Hutton RA. Thrombasthenia. Brit J Haematol 1974 10 371-387. 

Hagen I, Nurden A, Bjenum OJ, et al. Immunochemical evidence for protein alMioimalities in platelets from patients with Glanzmann s thrombasthenia and Bernard Soulier Syndrome. J Clin Invest 1980 65 722-731. 

Phillips DR, Agin PP. Platelet memfarine defects in Glanznunn s thrombasthenia. Evidence decr ised amounts of two ms glyoqiroteins. J Clin Invest 1977 60 535-545. 

ShulmanS,Kaq>addnS. Grossed immunoeleGtth esis of human platelet membranes Diminution of major antigens in Glananann s thrombasthenia and Bernard Soulier Syndrome. J Biol Chem 1980 255 4320-4327. 

Komedd E, NiewiarowsU S, Morinelli TA, et aL Effect of chymotrypsin and adenosine diphosphate on the exposure of fibrinogen receptors oi normal human and Glanamann s thrombasthenia platelets J Biol Chem 1981 256 5696-5701. 

Basani RB, Vilaire O, Shattil SJ, et al. Glanamann thrombasthenia due to a two amino acid deletion in the fourth calcium binding domain of alpha lib Demonstratiem of the importance of calcium binding domains in the conformation of alphallbbeta3. Blood 1996 88 167-173. 

CoUer BS, Cheredi DA, Asch E, Seligsohn U. Platelet vitronectin receptor expression differentiates Iraqi-Jewish from ArA patients wiAGIanamann thrombasthenia in Israel. Blood 1991 77 7S-83. 

Komedd E, Niewiarowski S, Morindli TA, et al. Effect of diymotrypsin and adenosine diphos diate on die exposure offibiinogen receptors on nnmal human and Glan2mann s thrombasthenia platelets. JBiol Chem 1981 256 5696-5701. 

A platelet function disorder, termed thrombasthenia, has been described in a Thoroughbred foal (Miura et al. 1987). The disorder is characterized by bleeding tendencies, absence of clot retraction, and lack of aggregation in response to ADP, collagen, and thrombin. 

Type I Glanzmann s thrombasthenia has been described in a female Great Pyrenees (Boudreaux et al. 1996a). Platelets have minimal aggregation in resporrse to ADP, collagen, thrombin, and PAF, and clot retraction is severely impaired. Surface expression of glycoprotein Ilbllla is markedly reduced. Therefore, this disorder closely resembles human type I Glanzmann s thrombasthenia. 

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