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Deprecated: Implicit conversion from float 267.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Blood+Rev 2020 ; 39 (ä): 100610 Nephropedia Template TP
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The role of beta-2-glycoprotein I in health and disease associating structure with function: More than just APS #MMPMID31471128
McDonnell T; Wincup C; Buchholz I; Pericleous C; Giles I; Ripoll V; Cohen H; Delcea M; Rahman A
Blood Rev 2020[Jan]; 39 (ä): 100610 PMID31471128show ga
Beta-2-Glycoprotein I (beta2GPI) plays a number of essential roles throughout the body. beta2GPI, C-reactive protein and thrombomodulin are the only three proteins that possess the dual capability to up and down regulate the complement and coagulation systems depending upon external stimulus. Clinically, beta2GPI is the primary antigen in the autoimmune condition antiphospholipid syndrome (APS), which is typically characterised by pregnancy morbidity and vascular thrombosis. This protein is also capable of adopting at least two distinct structural forms, but it has been argued that several other intermediate forms may exist. Thus, beta2GPI is a unique protein with a key role in haemostasis, homeostasis and immunity. In this review, we examine the genetics, structure and function of beta2GPI in the body and how these factors may influence its contribution to disease pathogenesis. We also consider the clinical implications of beta2GPI in the diagnosis of APS and as a potentially novel therapeutic target.