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      The role of beta-2-glycoprotein I in health and disease associating structure with function: More than just APS

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          Abstract

          Beta-2-Glycoprotein I (β2GPI) plays a number of essential roles throughout the body. β2GPI, 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, β2GPI 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, β2GPI is a unique protein with a key role in haemostasis, homeostasis and immunity. In this review, we examine the genetics, structure and function of β2GPI in the body and how these factors may influence its contribution to disease pathogenesis. We also consider the clinical implications of β2GPI in the diagnosis of APS and as a potentially novel therapeutic target.

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          International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS).

          S Miyakis, M Lockshin, T Atsumi … (2006)
          New clinical, laboratory and experimental insights, since the 1999 publication of the Sapporo preliminary classification criteria for antiphospholipid syndrome (APS), had been addressed at a workshop in Sydney, Australia, before the Eleventh International Congress on antiphospholipid antibodies. In this document, we appraise the existing evidence on clinical and laboratory features of APS addressed during the forum. Based on this, we propose amendments to the Sapporo criteria. We also provide definitions on features of APS that were not included in the updated criteria.
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            Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients.

            Ricard Cervera, Jean-Charles Piette, Josep Font … (2002)
            To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression. The clinical and serologic features of APS (Sapporo preliminary criteria) in 1,000 patients from 13 European countries were analyzed using a computerized database. The cohort consisted of 820 female patients (82.0%) and 180 male patients (18.0%) with a mean +/- SD age of 42 +/- 14 years at study entry. "Primary" APS was present in 53.1% of the patients; APS was associated with systemic lupus erythematosus (SLE) in 36.2%, with lupus-like syndrome in 5.0%, and with other diseases in 5.9%. A variety of thrombotic manifestations affecting the majority of organs were recorded. A catastrophic APS occurred in 0.8% of the patients. Patients with APS associated with SLE had more episodes of arthritis and livedo reticularis, and more frequently exhibited thrombocytopenia and leukopenia. Female patients had a higher frequency of arthritis, livedo reticularis, and migraine. Male patients had a higher frequency of myocardial infarction, epilepsy, and arterial thrombosis in the lower legs and feet. In 28 patients (2.8%), disease onset occurred before age 15; these patients had more episodes of chorea and jugular vein thrombosis than the remaining patients. In 127 patients (12.7%), disease onset occurred after age 50; most of these patients were men. These patients had a higher frequency of stroke and angina pectoris, but a lower frequency of livedo reticularis, than the remaining patients. APS may affect any organ of the body and display a broad spectrum of manifestations. An association with SLE, the patient's sex, and the patient's age at disease onset can modify the disease expression and define specific subsets of APS.
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              Glycans in the immune system and The Altered Glycan Theory of Autoimmunity: a critical review.

              Emanual Maverakis, Kyoungmi Kim, Michiko Shimoda … (2015)
              Herein we will review the role of glycans in the immune system. Specific topics covered include: the glycosylation sites of IgE, IgM, IgD, IgE, IgA, and IgG; how glycans can encode "self" identity by functioning as either danger associated molecular patterns (DAMPs) or self-associated molecular patterns (SAMPs); the role of glycans as markers of protein integrity and age; how the glycocalyx can dictate the migration pattern of immune cells; and how the combination of Fc N-glycans and Ig isotype dictate the effector function of immunoglobulins. We speculate that the latter may be responsible for the well-documented association between alterations of the serum glycome and autoimmunity. Due to technological limitations, the extent of these autoimmune-associated glycan alterations and their role in disease pathophysiology has not been fully elucidated. Thus, we also review the current technologies available for glycan analysis, placing an emphasis on Multiple Reaction Monitoring (MRM), a rapid high-throughput technology that has great potential for glycan biomarker research. Finally, we put forth The Altered Glycan Theory of Autoimmunity, which states that each autoimmune disease will have a unique glycan signature characterized by the site-specific relative abundances of individual glycan structures on immune cells and extracellular proteins, especially the site-specific glycosylation patterns of the different immunoglobulin(Ig) classes and subclasses.
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                Author and article information

                Contributors
                Thomas McDonnell
                Journal
                Journal ID (nlm-ta): Blood Rev
                Journal ID (iso-abbrev): Blood Rev
                Title: Blood Reviews
                Publisher: Elsevier
                ISSN (Print): 0268-960X
                ISSN (Electronic): 1532-1681
                Publication date PMC-release: 1 January 2020
                Publication date (Print): January 2020
                Volume: 39
                Electronic Location Identifier: 100610
                Affiliations
                [a ]Rheumatology, Division of Medicine, Rayne Institute, University College London, UK
                [b ]Nanostructure Group, Institute of Biochemistry, University of Greifswald, Germany
                [c ]Imperial College London, Imperial College Vascular Sciences, National Heart & Lung Institute, ICTEM, Hammersmith Campus, Du Cane Road, London, UK
                [d ]Department of Haematology, University College London Hospitals NHS Foundation Trust, London, UK
                Author notes
                [* ]Corresponding author. Thomas.mcdonnell.11@ 123456ucl.ac.uk
                Article
                Publisher ID: S0268-960X(19)30026-8 Publisher ID: 100610
                DOI: 10.1016/j.blre.2019.100610
                PMC ID: 7014586
                PubMed ID: 31471128
                SO-VID: 670d8bbd-f018-43b8-b4c6-fafa570dca14
                Copyright © © 2019 The Authors
                License:

                This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

                History
                Categories
                Subject: Article

                Keywords: beta-2-glycoprotein i,antiphospholipid syndrome,coagulation,complement,structural biochemistry
                Data availability:
                Keywords: beta-2-glycoprotein i, antiphospholipid syndrome, coagulation, complement, structural biochemistry

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