Fragility and structure of hemoglobin S fibers and gels and their consequences for gelation kinetics and rheology
Fragility and structure of hemoglobin S fibers and gels and their consequences for gelation kinetics and rheology
复制标题
血红蛋白 S 纤维和凝胶的脆性和结构及其对凝胶动力学和流变学的影响
DOI：
10.1182/blood.v83.2.573.bloodjournal832573
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发表时间：
1994
期刊：
影响因子：
20.3
通讯作者：
A. E. Guzman
中科院分区：
文献类型：
--
作者：
R. Briehl;A. E. Guzman
Pathogenesis in sickle cell disease depends on whether red blood cells can pass the microvasculature during the delay time before hemoglobin S gelation and cell rigidification occur. Here we observe individual hemoglobin S fibers by differential interference contrast (DIC) microscopy and show that hemoglobin S gels and fibers are fragile and easily broken by mechanical perturbation, and that breakage results in vast acceleration of gelation kinetics due to the creation of new, growing fiber-ends. Hence, in vivo this may be an important factor, in addition to hemoglobin concentration and degree of deoxygenation, that governs delay time and pathogenesis. Pathogenesis also depends on gel rheology and cell rigidification, which depend on fiber cross-linking. We show different mechanisms by which X-shaped, Y-shaped, and "zippering" cross-links form. Finally, we estimate the "on" rate constant for fiber growth to be about 200 mmol/(L.s) and obtain a value for the heterogeneous nucleation rate at 13.5 mmol/L heme.
镰状细胞血红蛋白双链的多态性组装。
影响因子：
5.6
作者：
Wellems,TE;Vassar,RJ;Josephs,R
通讯作者：
Josephs,R
镰状血红蛋白聚合的空间依赖性的理论描述。
影响因子：
3.4
作者：
Zhou,HX;Ferrone,FA
通讯作者：
Ferrone,FA