Lupus anticoagulant in cardiovascular diseases: the role of beta2-glycoprotein I.
The presence of antiphospholipid antibodies (aPL) in plasma is an important risk factor for the development of thrombosis. A major breakthrough came in 1990 with the finding that aPL do not react with phospholipids per se, but are directed towards beta2-glycoprotein I, a plasma protein with affinity for anionic phospholipids. Afterwards other plasma proteins with affinity for phospholipids have been identified as possible antigens for aPL. Human beta2-glycoprotein I is a heavily glycosylated plasma protein that has been implicated in the binding of aPL to negatively charged phospholipids. We recently solved the crystal structure of beta2-glycoprotein I. In this review we will discuss what the 3-dimensional structure teaches us about the role of beta2-glycoprotein I in the pathogenesis of the thrombotic complications characteristic of the antiphospholipid syndrome.
de Groot
P G
PG
Department of Haematology, University Medical Center, Utrecht, The Netherlands. ph.g.degroot@lab.azu.nl
Bouma
B
B
Lutters
B C
BC
Derksen
R H
RH
D000818:Animals; D017153:Antibodies, Anticardiolipin / Q000276:immunology / Q000502:physiology*; D002318:Cardiovascular Diseases / Q000503:physiopathology*; D000939:Epitopes; D006023:Glycoproteins / Q000502:physiology*; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000276:immunology / Q000502:physiology*; D008562:Membrane Glycoproteins / Q000502:physiology*; D013927:Thrombosis / Q000503:physiopathology; D053482:beta 2-Glycoprotein I
D017153:Antibodies, Anticardiolipin; D000939:Epitopes; D006023:Glycoproteins; D016682:Lupus Coagulation Inhibitor; D008562:Membrane Glycoproteins; D053482:beta 2-Glycoprotein I