Congenital Deficiency of Alpha-2-Adrenoceptors on Human Platelets : Description of Two Cases

Summary

The biochemistry and functionality of platelets from two related subjects (mother and son) with alpha-2-adrenoceptor-deficient platelets has been evaluated. Radioligand binding experimentes with the specific alpha-2-adrenergic-receptor antagonist, ³H-yohimbine, showed a drastic reduction of alpha-2-adrenoceptors in platelets from both subjects in comparison with the control values. Electron microscopy studies revealed a normal morphology and a normal number of alpha granules and dense bodies. Levels of adenine nucleotides; 5-hydroxytryptamine; B-thromboglobulin; platelet-factor-4 and thromboxane A₂ production were within normal limits.

Platelet aggregation and 5-hydroxytryplamine production in response to adrenalin (at concentrations up to 50 μM) were absent, whereas ADP, AA, PAF, collagen and thrombin-induced aggregation, secretion, Ca⁺⁺ flux and thromboxane A₂ production were normal.

The inhibitory effect caused by different concentrations of prostacyclin on Ca⁺⁺ flux, aggregation, secretion and thromboxane A₂ production of platelet functionally lacking of alpha-2-adrenoceptor was not distinguishable from control platelets and platelets preincubated with yohimbine.

Keywords

Alpha-2-adrenoceptors - Human platelets - Congenital deficiency

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