1. Osmotic gradient ektacytometry of red blood cells with varying degrees of spectrin deficiency. In the spectrin-deficient cells, the minimum deformability index observed in the hypotonic region (thin arrow) is shifted to the right of the control (shaded area), indicating a decrease in the cell surface area-to-volume ratio. The maximum deformability index (DImax) associated with the spectrin-deficient cells (thick arrow) is less than that of control cells, implying reduced surface area. The more pronounced the spectrin deficiency, the greater is the loss of surface area and the lower is the DImax. The osmolality in the hyperosmolar region at which the DI reaches half its maximum value is a measure of the hydration state of the red cells. It is decreased in the patient with the lowest spectrin content, indicating cellular dehydration. (Adapted from Chasis, Agre, and Mohandas.585 Used by permission.)
Source: Hereditary Spherocytosis and Hereditary Elliptocytosis, The Online Metabolic and Molecular Bases of Inherited Disease
Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular Bases of Inherited Disease; 2014 Available at: Accessed: October 07, 2017
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