| Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2013: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2012: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2011: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
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| Research Abstract |
We identified and characterized the juxtamembrane domain mutations of the GPIIb/IIIa receptor as a novel etiology of congenital macrothrombocytopenia. Because this domain is important for the inactive conformation the receptor, GPIIb/IIIa become constitutively activated by the mutation without ligand binding. The surface expression of platelet GPIIb/IIIa is decreased. There is spontaneous binding of PAC1 antibody, which recognizes active conformation of GPIIb/IIIa, to resting platelets without P-selectin expression. Large and decreased platelets were produced from the mutant GPIIb/IIIa-transduced megakaryocytes. This condition is the third most common cause of congenital macrothrombocytopenia in Japan.
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