| Literature DB >> 3198118 |
N V Cong1, G Uzan, M S Gross, C Jegou-Foubert, P Frachet, C Boucheix, G Marguerie, J Frézal.
Abstract
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 alpha subunit), which consists of two disulfide-linked heavy and light chains, and GPIIIa (the beta 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-bp GP2B (GPIIb) cDNA probe prepared from human megakaryocytes.Entities:
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Year: 1988 PMID: 3198118 DOI: 10.1007/bf00273658
Source DB: PubMed Journal: Hum Genet ISSN: 0340-6717 Impact factor: 4.132