Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P00750 (PLA)
 16,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Six cases of Glanzmann's thrombasthenia were studied using a platelet indirect radioactive Coombs (PIRC). In serum of two among six patients, an antibody was found, which reacted positively with all platelets except those of thrombasthenic patients. Such "anti-public" antibody which shortens the life of transfused platelets is a very serious complication of Glanzmann's thrombasthenia. Attempts to define the PLA group of the six Glanzmann patients, with human allo antisera recognizing PLA1 antigen, gave negative results. Three hypothesis were discussed: (1) Interference in the test of the aggregation defect of thrombasthenic platelets. However, anti-HLA antibodies were normally fixed in the PIRC test. (2) PLA2 gene and Glanzmann gene have a strong gametic association and all Glanzmann's patients are PLA1 negative. (3) Glanzmann gene is coding for the PLA gene substrate. In such case, thrombasthenic patients might be genetically PLA1 positive and phenotypically PLA1 negative. Moreover they would also be PLA2 negative, this could not be tested due to the lack of anti-PLA2 antiserum.
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PMID:[Glanzmann thrombasthenia, PLA1 antigen and anti-Glanzmann antibody]. 75 43

This study was undertaken to determine the role of platelet glycoprotein (GP) IIb/IIIa receptors in the modulation of plasminogen activator type-1 (PAI-1) release from human platelets as compared to other platelet functions. To address this issue, the effect of various agonists on human platelet aggregation, [125I]fibrinogen binding and the release of PAI-1 was examined in normal and Glanzmann's thrombasthenic (GT) platelets. In control subjects, maximum platelet aggregation and PAI-1 secretion were observed within 5 min in response to the different agonists including thrombin, collagen, adenosine diphosphate (ADP), and arachidonic acid. Agonist-induced platelet GpIIb/IIIa receptor activation was confirmed by [125I]fibrinogen binding analysis. In contrast, platelets from GT subjects demonstrated a lack of fibrinogen binding and a lack of an aggregatory response to all agonists tested except to the GPIb- mediated aggregation induced by ristocetin. However, GT platelets demonstrated normal responsiveness in secreting PAI-1 in response to the various agonists. Similarly, when platelet GpIIb/IIIa receptors were blocked in normal platelets by the tripeptide Arg-Gly-Asp (RGD) or the tetrapeptide Arg-Gly-Asp-Ser (RGDS) at 10(-3) M, agonist-induced platelet aggregation and fibrinogen binding were blocked, but platelet PAI-1 release was not blocked. Furthermore, flow cytometric analysis using dual fluorescence markers for the platelet GPIIb/IIIa membrane receptors (FITC-labeled cyclic RGD analog, XL086) and for the alpha granule (PE-monoclonal antibody for P-selectin) demonstrated a dissociation between the platelet GPIIb/IIIa receptors and granular secretion. These results suggest a lack of a role for platelet GpIIb/IIIa receptors in the modulation of platelet PAI-1 release.
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PMID:Role of platelet GpIIb/IIIa receptors in the modulation of platelet plasminogen activator inhibitors type-1 (PAI-1) release. 815 39