Regulation of Na-K-2Cl Cotransport in Red Cells

Flatman, Peter W.

doi:10.1007/0-387-23752-6_7

Cited by 4 publications

(1 citation statement)

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“…Therefore, strategies that prevent cellular dehydration should be explored for the treatment of SCD. The inhibition of potassium-chloride cotransport, in which potassium causes the movement of chloride ions and water, produces an osmotic imbalance and causes dehydration, with further polymerization of HbS [64]. The calciumactivated potassium channel known as the Gardos channel is also present on sickle erythrocytes and could be inhibited to promote an adequate osmotic balance [65].…”

Section: Inhibition Of Erythrocyte Dehydrationmentioning

confidence: 99%

Sickle Cell Disease – Current Treatment and New Therapeutical Approaches

Melo¹,

Ercolin²,

Chelucci³

et al. 2015

Inherited Hemoglobin Disorders

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Sickle cell disease (SCD) is one of the most common genetic disorders worldwide. It is caused by a point mutation that changes glutamic acid (Glu6) to valine (Val6) in the β chain of hemoglobin. Vaso-occlusion is the most well-known problem associated with SCD. Despite recent advances in understanding the disease at the molecular level, few therapeutic strategies are available. Hydroxyurea is the only drug currently approved by the U.S. Food and Drug Administration for the disease, and it has serious adverse effects and lack of efficacy in some patients. However, new therapeutic approaches are under investigation in the hope of discovering new drugs to treat SCD.These include agents that: a) increase nitric oxide bioavailability; b) modify the rheological properties of the blood; c) bind covalently to hemoglobin; d) prevent hemoglobin dehydration; e) reduce iron overload; and f) induce the expression of gamma globin and fetal hemoglobin. In this chapter, we discuss the current treatment of SCD and the advances made in medicinal chemistry to find new drugs to treat this neglected hematological disease.

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Section: Inhibition Of Erythrocyte Dehydrationmentioning

confidence: 99%