The role of beta-2-glycoprotein I in health and disease associating structure with function: More than just APS.
Antiphospholipid syndrome
Beta-2-glycoprotein I
Coagulation
Complement
Structural biochemistry
Journal
Blood reviews
ISSN: 1532-1681
Titre abrégé: Blood Rev
Pays: England
ID NLM: 8708558
Informations de publication
Date de publication:
01 2020
01 2020
Historique:
received:
07
03
2019
revised:
09
08
2019
accepted:
15
08
2019
pubmed:
1
9
2019
medline:
24
9
2020
entrez:
1
9
2019
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 31471128
pii: S0268-960X(19)30026-8
doi: 10.1016/j.blre.2019.100610
pmc: PMC7014586
pii:
doi:
Substances chimiques
beta 2-Glycoprotein I
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
100610Subventions
Organisme : MRF
ID : MRF_MRF-057-0004-RG-MCDO-C0800
Pays : United Kingdom
Organisme : Versus Arthritis
ID : 19423
Pays : United Kingdom
Organisme : Medical Research Council
ID : MR/P017371/1
Pays : United Kingdom
Organisme : Versus Arthritis
ID : 21223
Pays : United Kingdom
Organisme : Versus Arthritis
ID : 21992
Pays : United Kingdom
Informations de copyright
Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of Competing Interest TM, CP, IG and AR are inventors on the patent for Domain I.
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