Charache S, Conley C L, Waugh D F, Ugoretz R J, Spurrell J R
J Clin Invest. 1967 Nov;46(11):1795-811. doi: 10.1172/JCI105670.
Hemoglobin C is less soluble than hemoglobin A in red cells, in hemolysates, and in dilute phosphate buffer. Its relative insolubility may be explained by electrostatic interactions between positively charged beta6-lysyl groups and negatively charged groups on adjacent molecules. Red cells from patients with homozygous hemoglobin C (CC) disease exhibit aberrant physical properties which suggest that the cells are more rigid than normal erythrocytes. They pass through membrane filters less readily than normal red cells do, and their viscosity is higher than that of normal cells. Differences from normal cells are exaggerated if mean corpuscular hemoglobin concentration (MCHC) is increased, by suspension in hypertonic salt solution. Increased rigidity of CC cells, by accelerating their fragmentation, may be responsible for formation of microspherocytes. These small dense cells are exceptionally rigid, and probably are even more susceptible to fragmentation and sequestration. Rigidity of CC cells can be attributed to a "precrystalline" state of intracellular hemoglobin, in which crystallization does not occur, although the MCHC exceeds the solubility of hemoglobin in hemolysates.
血红蛋白C在红细胞、溶血产物及稀磷酸盐缓冲液中的溶解度低于血红蛋白A。其相对不溶性可通过带正电荷的β6-赖氨酰基团与相邻分子上带负电荷基团之间的静电相互作用来解释。纯合血红蛋白C（CC）病患者的红细胞表现出异常的物理特性，这表明这些细胞比正常红细胞更僵硬。它们通过膜过滤器的能力不如正常红细胞，且其粘度高于正常细胞。如果通过悬浮在高渗盐溶液中提高平均红细胞血红蛋白浓度（MCHC），与正常细胞的差异会更加明显。CC细胞硬度增加，加速了其破碎，可能是导致微球形红细胞形成的原因。这些小而致密的细胞异常僵硬，可能甚至更容易破碎和被隔离。CC细胞的僵硬可归因于细胞内血红蛋白的“预结晶”状态，尽管MCHC超过了血红蛋白在溶血产物中的溶解度，但此时并未发生结晶。
