Abstract
Sickle cell disease is caused by the self assembly of deoxy-sickle hemoglobin (HbS) into fibers which rigidify and distort red cells. Fiber assembly is auto-catalytic in that the surface of existing fibers acts as a nucleating template for the assembly of new fibers (heterogeneous nucleation). Thus fibers are found typically as bundles of aligned particles. Understanding fiber-fiber interaction is crucial to understanding the pathology of fiber formation. Moreover, disrupting fiber-fiber interactions would likely ameliorate the severity of the disease. We have obtained images of both laterally associated and isolated fibers by cryo-electron microscopy (Figure 1). Optical diffraction indicates that laterally associated fibers tend to have much better long range order than isolated fibers. This observation suggests that deoxy-HbS fibers are more disordered when they are not mechanically coupled by inter-fiber contacts. In this study we assess the effects of fiber-fiber interactions on torsional disorder of deoxy-HbS fibers.
Published Version
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