Waugh R E
Biophys J. 1987 Mar;51(3):363-9. doi: 10.1016/S0006-3495(87)83358-1.
Several workers have identified molecular abnormalities associated with inherited blood disorders. The present work examines how these alterations in molecular structure affect the viscoelastic properties of the red blood cell membrane. Changes in the membrane shear modulus, the membrane viscosity, and the apparent membrane bending stiffness were observed in cells of eight patients having a variety of disorders: Two had reductions in the number of high-affinity ankyrin binding sites, two had abnormalities associated with the protein band 4.1, and six were known to be deficient in spectrin. The data suggest that the membrane shear modulus is proportional to the density of spectrin on the membrane and support the view that spectrin is primarily responsible for membrane shear elasticity. Although membranes having abnormalities associated with the function of ankyrin or band 4.1 exhibited reduced elasticity, the degree of mechanical dysfunction was quantitatively inconsistent with the extent of the molecular abnormality. This indicates that these skeletal components do not play a primary role in determining membrane shear elasticity. The membrane viscosity was reduced in seven of the eight patients studied. The reduction in viscosity was usually greater than the reduction in shear modulus, but the degree of reduction in viscosity was variable and did not correlate well with the degree of molecular abnormality.
几位研究人员已经确定了与遗传性血液疾病相关的分子异常情况。目前的研究工作探讨了这些分子结构的改变如何影响红细胞膜的粘弹性特性。在患有各种疾病的8名患者的细胞中观察到了膜剪切模量、膜粘度和表观膜弯曲刚度的变化:2名患者的高亲和力锚蛋白结合位点数量减少,2名患者与带4.1蛋白相关存在异常,6名患者已知血影蛋白缺乏。数据表明,膜剪切模量与膜上血影蛋白的密度成正比,并支持血影蛋白主要负责膜剪切弹性的观点。尽管与锚蛋白或带4.1蛋白功能相关异常的膜表现出弹性降低,但机械功能障碍的程度在数量上与分子异常的程度不一致。这表明这些骨架成分在决定膜剪切弹性方面不发挥主要作用。在研究的8名患者中的7名患者中,膜粘度降低。粘度的降低通常大于剪切模量的降低,但粘度降低的程度是可变的,并且与分子异常的程度没有很好的相关性。