Ayi Kodjo, Turrini Franco, Piga Antonio, Arese Paolo
Dipartimento di Genetica, Biologia e Biochimica, Università di Torino, Italy.
Blood. 2004 Nov 15;104(10):3364-71. doi: 10.1182/blood-2003-11-3820. Epub 2004 Jul 27.
High frequency of erythrocyte (red blood cell [RBC]) genetic disorders such as sickle cell trait, thalassemia trait, homozygous hemoglobin C (Hb-C), and glucose-6-phosphate dehydrogenase (G6PD) deficiency in regions with high incidence of Plasmodium falciparum malaria and case-control studies support the protective role of those conditions. Protection has been attributed to defective parasite growth or to enhanced removal of the parasitized RBCs. We suggested enhanced phagocytosis of rings, the early intraerythrocytic form of the parasite, as an alternative explanation for protection in G6PD deficiency. We show here that P falciparum developed similarly in normal RBCs and in sickle trait, beta- and alpha-thalassemia trait, and HbH RBCs. We also show that membrane-bound hemichromes, autologous immunoglobulin G (IgG) and complement C3c fragments, aggregated band 3, and phagocytosis by human monocytes were remarkably higher in rings developing in all mutant RBCs considered except alpha-thalassemia trait. Phagocytosis of ring-parasitized mutant RBCs was predominantly complement mediated and very similar to phagocytosis of senescent or damaged normal RBCs. Trophozoite-parasitized normal and mutant RBCs were phagocytosed similarly in all conditions examined. Enhanced phagocytosis of ring-parasitized mutant RBCs may represent the common mechanism for malaria protection in nonimmune individuals affected by widespread RBC mutations, while individuals with alpha-thalassemia trait are likely protected by a different mechanism.
在恶性疟原虫疟疾高发地区,红细胞(红血球[RBC])遗传疾病如镰状细胞性状、地中海贫血性状、纯合血红蛋白C(Hb-C)和葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症的发生率很高,病例对照研究支持了这些病症的保护作用。这种保护作用归因于寄生虫生长缺陷或被寄生红细胞的清除增强。我们提出,对环状体(寄生虫早期红细胞内形态)吞噬作用增强是G6PD缺乏症具有保护作用的另一种解释。我们在此表明,恶性疟原虫在正常红细胞以及镰状性状、β和α地中海贫血性状以及HbH红细胞中发育情况相似。我们还表明,在除α地中海贫血性状外的所有所考虑的突变红细胞中发育的环状体中,膜结合半色素、自身免疫球蛋白G(IgG)和补体C3c片段、聚集的带3以及人类单核细胞的吞噬作用明显更高。被环状体寄生的突变红细胞的吞噬作用主要由补体介导,与衰老或受损正常红细胞的吞噬作用非常相似。在所有检测条件下,被滋养体寄生的正常和突变红细胞的吞噬情况相似。被环状体寄生的突变红细胞吞噬作用增强可能是受广泛红细胞突变影响的非免疫个体疟疾保护的共同机制,而具有α地中海贫血性状的个体可能通过不同机制得到保护。