Solid-like behaviour of unsheared sickle haemoglobin gels and the effects of shear

Robin W. Briehl

Research output: Contribution to journalArticle

27 Scopus citations

Abstract

Pathogenesis in sickle cell disease depends on the polymerization of deoxyhaemoglobin S into long fibres followed by 'gel' formation. The 'gelation' renders the affected erythrocytes less deformable than normal so that they obstruct the microvasculature and the 'gelation' process has been one of the targets for the development of therapeutic treatments of sickle cell disease. 'Gelation', however, acts through the rheological properties it induces and rheological abnormalities are therefore the immediate bases of pathogenesis. Although there has been very little study of the rheology of haemoglobin S haemolysates, the 'gels' are generally considered to be highly viscous and thixotropic on the basis of gross observation. Limited and generally qualitative observations show that high viscosity depends on deoxygenation1,2 and exhibits hysteresis in gelling/ungelling cycles1; also that shearing accelerates gelation3,4 and can induce formation of fibre aggregates and crystals in suspension which, in contrast to the 'gel', are fluid5. Using transient and steady state methods it is shown here that unsheared sickle deoxyhaemoglobin preparations are solid-like, consistent with a gellike nature, whereas shearing converts them to thixotropic viscous systems. These results underline the marked variation and thixotropy the system can undergo and may be relevant to the pathogenesis, clinical course and therapy of sickle cell disease.

Original languageEnglish (US)
Pages (from-to)622-624
Number of pages3
JournalNature
Volume288
Issue number5791
DOIs
StatePublished - Dec 1 1980

ASJC Scopus subject areas

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