Red cell dehydration

Some young sickle red cells undergo a fast process of dehydration after they enter the circulation. There are, in effect, intermediate dense cells and very dense cells. It is possible that different mechanisms are involved in each of these types. It is clear that Ca2+-dependent K+ efflux and probably deoxy-induced K+ efflux are responsible for the latter, and that K:Cl co-transport overexpression, due to the relative youth of the red cells, is responsible for the former. K:Cl is no longer active in normal adult red cells, while reticulocytes retain a modicum of this system. Finally, cycles of oxy-deoxygenation seem

Strand 1

Fig. 14.6 Polymer Hb S structure

This is the assembly unit of the Hb S polymer: the Werner-Love double strand. This double strand is part of the Hb S crystal, but in the crystal it propagates in all directions to form a lattice. The sickle fiber wraps around to form a helical fiber. The squares depict the four different areas of contact that form the double strand.

Strand 1

Strand 2

to be required for dense cell formation. Patients with sickle cell anemia who have high Hb F levels and coexistent a thalassemia have fewer dense cells. Dense cells and ISCs both have very short lives (probably less than 5 days) and have markedly right-shifted oxygen equilibrium curves.

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