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Polymerization and Sickle Cell Disease: A Molecular View
Author(s) -
FERRONE FRANK A.
Publication year - 2004
Publication title -
microcirculation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.793
H-Index - 83
eISSN - 1549-8719
pISSN - 1073-9688
DOI - 10.1080/10739680490278312
Subject(s) - polymerization , mechanism (biology) , disease , rheology , chemistry , biophysics , medicine , polymer , biology , philosophy , physics , thermodynamics , epistemology , pathology , organic chemistry
The present molecular‐level understanding of polymerization and sickling is reviewed for 2 central questions in sickle hemoglobin pathophysiology, viz., what determines when cells sickle, and what determines when cells get stuck. The description of sickling includes the central aspects of the double nucleation mechanism, as well as recent results on the effects of crowding, with an emphasis on the physiological applicability of this fundamental knowledge. In considering when cells get stuck, new measurements of individual fiber stiffness and the processes of depolymerization are also considered. Finally, a fundamental connection is shown between thermodynamics and rheology.