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Lupus
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Review : β2-Glycoprotein I as a 'Cofactor' for anti-phospholipid reactivity with endothelial cells

PL Meroni

Department of Internal Medicine, IRCCS Policlinico, University of Milan

N. Del Papa

Department of Internal Medicine, IRCCS Policlinico, University of Milan

E. Raschi

Department of Internal Medicine, IRCCS Policlinico, University of Milan

P. Panzeri

Department of Internal Medicine, IRCCS Policlinico, University of Milan

MO Borghi

Department of Internal Medicine, IRCCS Policlinico, University of Milan

A. Tincani

Department of Internal Medicine, IRCCS Policlinico, University of Milan

G. Balestrieri

Servizio di Immunologia Clinica, Spedali Civili, Brescia, Italy

MA Khamashta

Lupus Research Unit, St Thomas' Hospital, London, UK

Grv Hughes

Lupus Research Unit, St Thomas' Hospital, London, UK

T. Koike

Department of Medicine , Hokkaido University, Sapporo, Japan

SA Krilis

Department of Immunology, Allergy and Infectious Diseases, The St George Hospital, University of South Wales School of Medicine, Kogarah, Australia

β2-glycoprotein I (β2GPI) is a cofactor for anti-phospholipid (aPL) binding to cardiolipin (CL)- coated plates. β2GPI is also able to bind to endothelial cell (EC) membranes as supported by in-vivo as well as by in-vitro studies. The PL-binding site in the fifth domain of the molecule is involved in the adhesion to endothelium. Actually, specific mutations in this molecular portion abolish endothelium binding and a synthetic peptide spanning the sequence Glu274-Cys288 of the CL binding site displays comparable adhesion to EC monolayers. Heparan sulphate appears to be one of the anionic EC membrane structures with which cationic β2GPI interacts, as supported by studies with heparitinase-treated EC. β2GPI binding to EC might be related to its activity as endothelial growth factor or as a lipid-carrying glycoprotein. Adhesion of β2GPI to endothelial membranes offers suitable epitopes for circulating aPL that, once bound, can induce cell activation

Key Words: anti-phospholipid antibodies • β2-glycoprotein I • endothelial cells • heparan sulphate

Lupus, Vol. 7, No. 2 suppl, S44-S47 (1998)
DOI: 10.1177/096120339800700211


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