Carlson J, Nash G B, Gabutti V, al-Yaman F, Wahlgren M
Microbiology and Tumor Biology Center, Karolinska Institute, Stockholm, Sweden.
Blood. 1994 Dec 1;84(11):3909-14.
Genes for two lethal diseases, thalassemia and sickle cell anemia, are favored by evolution because, in their heterozygous form, they protect against cerebral malaria. Rosette formation, the binding of uninfected red cells (RBCs) to Plasmodium falciparum-infected RBCs (PRBCs), has previously been found to be associated with cerebral malaria, the most important severe manifestation of P falciparum malaria. We show here that thalassemic RBCs and, under certain conditions, even hemoglobin S (HbS)-containing RBCs possess an impaired ability to bind to PRBCs, forming small and weak erythrocyte rosettes compared with rosettes formed by normal RBCs. This decreased rosetting ability is associated with the small size of the thalassemic RBCs and with distortion of the mechanical properties of HbS-containing RBCs. The impairment of rosette formation may hinder the development of cerebral malaria by abatement of sequestration.
两种致命疾病——地中海贫血和镰状细胞贫血的基因受到进化的青睐,因为在杂合形式下,它们能预防脑型疟疾。红细胞花结形成,即未感染的红细胞(RBC)与恶性疟原虫感染的红细胞(PRBC)结合,此前已被发现与脑型疟疾有关,脑型疟疾是恶性疟最重要的严重表现形式。我们在此表明,地中海贫血患者的红细胞,以及在某些情况下,甚至含有血红蛋白S(HbS)的红细胞,与正常红细胞形成的花结相比,它们与PRBC结合的能力受损,形成的红细胞花结小且弱。这种花结形成能力的下降与地中海贫血患者红细胞的小尺寸以及含有HbS的红细胞力学性能的扭曲有关。花结形成的受损可能通过减少滞留来阻碍脑型疟疾的发展。