Ma Xiaohui, Zhao Zhen, Zhang Yan, Li Lingbo, Zhong Jing
Cancer Center, The First Hospital of Jilin University, Changchun, China.
Department of Blood Transfusion, The First Hospital of Jilin University, Changchun, China.
Clin Appl Thromb Hemost. 2024 Jan-Dec;30:10760296241309638. doi: 10.1177/10760296241309638.
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.
诺普斯血型系统是国际输血协会（ISBT）于1992年认可的一个独立血型系统，最新发现它由13种抗原组成，这些抗原存在于一种含2489个氨基酸的糖蛋白上，该糖蛋白被称为补体C3b/C4b受体1（CR1）。红细胞KN抗原于1970年首次被报道，CR1是一个蛋白质编码基因，是补体激活受体（RCA）家族的成员之一，位于1号染色体的“RCA簇”区域。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，其表达的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。针对诺普斯系统的抗体通常不结合补体，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诺普斯抗体。不过，一般来说，它们仅通过直接抗球蛋白试验（DAT）呈阳性反应，且大多数不会导致新生儿溶血病（HDN）。本文是对诺普斯血型系统研究进展的综述。
