Eyler Christine E, Jackson Terry, Elliott Laine E, De Castro Laura M, Jonassaint Jude, Ashley-Koch Allison, Telen Marilyn J
Division of Hematology and Centre for Human Genetics, Department of Medicine, Duke Comprehensive Sickle Cell Centre, Duke University, Durham, NC 27710, USA.
Br J Haematol. 2008 Apr;141(1):105-8. doi: 10.1111/j.1365-2141.2008.07008.x.
Sickle red cell (SS RBC) adhesion is thought to contribute to sickle cell disease (SCD) pathophysiology. SS RBC adhesion to laminin increases in response to adrenaline stimulation of beta(2)-adrenergic receptors (beta(2)ARs) and adenylate cyclase (ADCY6), and previous evidence suggests such activation occurs in vivo. We explored whether polymorphisms of the beta(2)AR and ADCY6 genes (ADRB2 and ADCY6, respectively) affect RBC adhesion to laminin. We found that the beta(2)AR arg(16)-->gly substitution and two non-coding ADCY6 polymorphisms were associated with elevated adhesion. We postulate that ADRB2 and ADCY6 polymorphisms may influence SCD severity through the mechanism of RBC adhesion.
镰状红细胞(SS RBC)黏附被认为与镰状细胞病(SCD)的病理生理学有关。SS RBC对层粘连蛋白的黏附会因肾上腺素刺激β₂ - 肾上腺素能受体(β₂ARs)和腺苷酸环化酶(ADCY6)而增加,先前的证据表明这种激活在体内会发生。我们探究了β₂AR和ADCY6基因(分别为ADRB2和ADCY6)的多态性是否会影响RBC对层粘连蛋白的黏附。我们发现β₂AR的精氨酸(16)→甘氨酸替代以及两个非编码ADCY6多态性与黏附增加有关。我们推测ADRB2和ADCY6多态性可能通过RBC黏附机制影响SCD的严重程度。