The Sickle-Cell Fiber Revisited

被引:6
作者
Bishop, Marilyn F. [1 ]
Ferrone, Frank A. [2 ]
机构
[1] Virginia Commonwealth Univ, Dept Phys, Richmond, VA 23284 USA
[2] Drexel Univ, Dept Phys, Philadelphia, PA 19104 USA
关键词
double strand; electron microscopy; sickle cell polymer; REFINED CRYSTAL-STRUCTURE; HEMOGLOBIN-S; DEOXYHEMOGLOBIN-S; HETEROGENEOUS NUCLEATION; INTERMOLECULAR CONTACTS; STRUCTURAL-ANALYSIS; POLYMERIZATION; RESIDUES; MUTANT; RECONSTRUCTION;
D O I
10.3390/biom13030413
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Sickle cell disease is the consequence of a single point mutation on the surface of the beta chains of the hemoglobin molecule leading to the formation of rigid polymers that disrupt circulation. It has long been established that the polymers are comprised of seven pairs of double strands that are twisted replicas of the double strands found in crystals. Here, we review several newer developments that elaborate on that simple model and provide deeper insights into the process.
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页数:10
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