| Autor: |
Nguyen van Cong, Uzan, G., Gross, M., Jegou-Foubert, C., Frachet, P., Boucheix, C., Marguerie, G., Frézal, J. |
| Zdroj: |
Human Genetics; 1988, Vol. 80 Issue 4, p389-392, 4p |
| Abstrakt: |
The platelet GPIIb-IIIa complex functions as a receptor for fibrinogen, fibronectin, and von Willebrand factor on activated platelets. This glycoprotein is a member of a broadly distributed family of structurally and immunologically related membrane receptors involved in cell-cell contact and cell-matrices interactions. GPIIb-IIIa is a heterodimer complex composed of GPIIb (the α subunit), which consists of two disulfide-linked heavy and light chains, and GPIIIa (the β subunit), which is a single polypeptide chain. Congenital absence of platelet GPIIb-IIIa in Glanzmann's thrombasthenia results in a severe bleeding disorder characterized by defective platelet aggregation and failure of fibrinogen to bind to platelets. The gene coding for GPIIb was located on 17q21.1-17q21.3 as determined by in situ hybridization with a 2650-pb GP2B (GPIIb) cDNA probe prepared from human megakaryocytes. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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