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Thromb Haemost 1997; 77(05): 0991-0995
DOI: 10.1055/s-0038-1656091
Platelets
Schattauer GmbH Stuttgart

Defective Signal Transduction through the Thromboxane A2 Receptor in a Patient with a Miid Bleeding Disorder: Deficiency of the Inositol 1,4,5-Triphosphate Formation despite Normal G-protein Activation

Tetsuo Mitsui
The Department of Pediatrics, Yamagata University, School of Medicine, lida-Nishi, Yamagata-city, Yamagata, Japan
,
Shinkichi Yokoyama
The Department of Pediatrics, Yamagata University, School of Medicine, lida-Nishi, Yamagata-city, Yamagata, Japan
,
Yukitoshi Shimizu
The Department of Pediatrics, Yamagata University, School of Medicine, lida-Nishi, Yamagata-city, Yamagata, Japan
,
Michihiko Katsuura
The Department of Pediatrics, Yamagata University, School of Medicine, lida-Nishi, Yamagata-city, Yamagata, Japan
,
Kaori Akiba
The Department of Pediatrics, Yamagata University, School of Medicine, lida-Nishi, Yamagata-city, Yamagata, Japan
,
Kiyoshi Hayasaka
The Department of Pediatrics, Yamagata University, School of Medicine, lida-Nishi, Yamagata-city, Yamagata, Japan
› Author Affiliations
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Publication History

Received 02 April 1996

Accepted after resubmission 29 January 1997

Publication Date:
11 July 2018 (online)

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Summary

We describe an 11-year-old girl with a mild bleeding disorder since early childhood. The disorder was characterized by a prolonged bleeding time, and the patient’s platelets showed defective aggregation responses to thromboxane A2 (TXA2) mimetic U46619 and arachidonic acid. In contrast, the platelets showed normal responses to thrombin and Ca ionophore A23187. When the platelet TXA2 receptor was examined with the [3H]-labeled TXA2 agonist U46619, the equilibrium dissociation rate constants (kd) and the maximal concentration of binding sites (Bmax) of the patient's platelets were within normal ranges. Normal GTPase activity was also induced in the patient's platelets by stimulation with U46619, however, inositol 1,4,5-triphosphate (IP3) formation was not induced by U46619. These results suggested that the patient’s platelets had a defect in phospholipase C activation beyond TXA2 receptors.

 

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