Xiaohui Ma, Zhen Zhao, Yan Zhang, Lingbo Li, Jing Zhong
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引用次数: 0
Abstract
The Knops blood group system is an independent blood group system recognized by International Society of Blood Transfusion (ISBT) in 1992, and latest time consisting of 13 antigens carried on a glycoprotein of 2489 amino acids and called the Complement C3b/C4b Receptor 1 (CR1). Erythrocyte KN antigen was first reported in 1970, and CR1 is a protein coding gene that is a member of the receptors of complement activation (RCA) family and is located in the "cluster RCA" region of chromosome 1. CR1 is an important participant in the erythrocyte immune machinery and plays an major role in inhibiting complement activation, and polymorphisms in its expression have been closely associated with a variety of diseases, including systemic lupus erythematosus (SLE), malaria, Plasmodium falciparum malaria, Alzheimer's disease (AD) and leprosy. Antibodies to the Knops system usually do not bind to complement and do not cause a hemolytic reaction. However, anti-Knops antibodies can be detected in the serum of some pregnant women. Generally, however, they only test positive by direct antiglobulin test (DAT) and most of them do not cause hemolytic disease of the newborn (HDN). This article is a review of the progress of the Knops blood group system.
Knops血型系统是1992年被国际输血学会(International Society of blood Transfusion, ISBT)认可的一个独立的血型系统,最新的血型系统是由携带有2489个氨基酸的糖蛋白的13种抗原组成,称为补体C3b/C4b受体1 (CR1)。红细胞KN抗原于1970年首次报道,CR1是一种蛋白质编码基因,是补体激活受体(receptor of complement activation, RCA)家族的成员,位于1号染色体的“簇RCA”区。CR1是红细胞免疫机制的重要参与者,在抑制补体激活中起主要作用,其表达多态性与多种疾病密切相关,包括系统性红斑狼疮(SLE)、疟疾、恶性疟原虫疟疾、阿尔茨海默病(AD)和麻风病。Knops系统的抗体通常不与补体结合,也不会引起溶血反应。然而,在一些孕妇的血清中可以检测到抗knops抗体。但一般只在直接抗球蛋白试验(DAT)中呈阳性,多数不会引起新生儿溶血病(HDN)。本文就Knops血型系统的研究进展作一综述。
期刊介绍:
CATH is a peer-reviewed bi-monthly journal that addresses the practical clinical and laboratory issues involved in managing bleeding and clotting disorders, especially those related to thrombosis, hemostasis, and vascular disorders. CATH covers clinical trials, studies on etiology, pathophysiology, diagnosis and treatment of thrombohemorrhagic disorders.