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Polymerization and sickle cell disease: a molecular view
Journal article   Peer reviewed

Polymerization and sickle cell disease: a molecular view

Frank A Ferrone
Microcirculation (New York, N.Y. 1994), v 11(2), pp 115-128
Mar 2004
PMID: 15280087

Abstract

Erythrocytes, Abnormal - chemistry Multiprotein Complexes - metabolism Thermodynamics Hemoglobin, Sickle - chemistry Multiprotein Complexes - chemistry Anemia, Sickle Cell - physiopathology Models, Chemical Anemia, Sickle Cell - metabolism Polymers - chemistry Erythrocytes, Abnormal - metabolism Polymers - metabolism Hemoglobin, Sickle - metabolism
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.

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Web of Science research areas
Hematology
Peripheral Vascular Disease
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