PATHOGENESIS OF HEMOLYTIC ANEMIA IN HOMOZYGOUS HEMOGLOBIN C DISEASE
PATHOGENESIS OF HEMOLYTIC ANEMIA IN HOMOZYGOUS HEMOGLOBIN C DISEASE
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DOI：
10.1172/jci105670
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发表时间：
1967-01-01
影响因子：
15.9
通讯作者：
SPURRELL, JR
中科院分区：
文献类型：
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作者：
CHARACHE, S;CONLEY, CL;SPURRELL, JR
血红蛋白C在红细胞、溶血产物和稀磷酸盐缓冲液中的溶解度低于血红蛋白A。它的相对不溶性可以通过带正电荷的[β]6-赖氨酰基团和相邻分子上带负电荷的基团之间的静电相互作用来解释。来自患有纯合血红蛋白C（CC）疾病的患者的红细胞表现出异常的物理性质，这表明细胞比正常红细胞更坚硬。它们比正常红细胞更不容易通过膜过滤器，并且它们的粘度高于正常细胞。如果通过在高渗盐溶液中悬浮使平均红细胞血红蛋白浓度（MCHC）增加，则与正常细胞的差异被夸大。CC细胞的刚性增加，通过加速其碎裂，可能是微球细胞形成的原因。这些小而致密的细胞非常坚硬，可能更容易破碎和隔离。CC细胞的刚性可归因于细胞内血红蛋白的“预结晶”状态，其中不发生结晶，尽管MCHC超过了血红蛋白在溶血产物中的溶解度。
Hemoglobin C is less soluble than hemoglobin A in red cells, inhemolysates, and in dilute phosphate buffer. Its relative insolubility may be explained by electrostatic interactions between positively charged [beta]6-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 hyper-tonic 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.