Author/Editor     Hojnik, Maja
Title     Imunogenost in patogenost različnih oblik beta2-glikoproteina I pri antifosfolipidnem sindromu
Translated title     Immunogenicity and pathogenicity of different forms of beta2- glycoprotein I in the antiphospholipid syndrome
Type     monografija
Place     Ljubljana
Publisher     Medicinska fakulteta, Ljubljana
Publication year     1997
Volume     str. 133
Language     slo, eng
Abstract     The studies presented in this thesis were aimed at clarifying the antigenic and possible immunogenic roles of beta2-glycoprotein I (beta2-GPI) in the antiphospholipid syndrome (APS). APS is a disorder characterized by the presence of antiphospholipid antibodies (aPL) and arterial or venous thrombosis, recurrent pregnancy loss or thrombocytopenia. Beta2-GPI has recently been recognized as the principal antigen bound in vitro by aPL. The protein consists of 5 homologous domains (I-V), it binds to anionic phospholipids and possesses anticoagulant properties. To dissect the significance of individual domains in providing antigenic and/or immunogenic epitopes, deletion mutants representing different sets of beta2-GPI domains were employed. The first chapter is a review article on the APS, and particularly, on the heart valve involvement in patients with aPL. This is a relatively common, but only recently perceived clinical association of aPL. It may explain the occurrence of Libman-Sacks endocarditis in patients with SLE, in about a third of patients with primary APS, as well as in apparently healthy individuals harboring aPL. Data on the prevalence, morphologic and functional types of aPL-related heart valve abnormalities, their clinical significance, possible complications, prevention and treatment are discussed. Proposed are also the conceivable pathogenetic mechanisms, which include platelet and endothelial cell activation or damage, enhanced blood coagulability, and deposition of antibodies along with complement components in the valve subendothelium. (Abstract truncated at 2000 character)
Descriptors     ANTIPHOSPHOLIPID SYNDROME
ANTIBODIES, ANTICARDIOLIPIN
GLYCOPROTEINS
MICE, INBRED BALB C
LUPUS ERYTHEMATOSUS, SYSTEMIC
ENDOCARDITIS
ANTIBODIES, MONOCLONAL
IGG
AUTOANTIBODIES