Biophysical Studies Aimed at Potential Therapies of Hemoglobin Associated Diseases

Abstract

Hemoglobin (Hb), the most abundant protein found in the red blood cell (RBC), carries gases like oxygen and carbon dioxide throughout the body. However, various conditions, both genetic and environmental, can impede its ability to perform this (and other) necessary functions. We studied some of the most imperative properties of Hb and the RBC as they pertain to sickle cell disease (SCD): oxygen affinity and RBC deformability. A Hemox Analyzer, a dual-wavelength spectrophotometer, was used to measure oxygen affinity in sRBC treated with a potential drug intended to improve oxygen affinity. Deformability, the ability of the cells to elongate when exposed to shear, was measured as a function of osmotic pressure in a refurbished Technicon Ektacytometer. This characteristic of the cells is extremely important, especially in SCD, as rigid cells 1) are unable to traverse the narrow capillaries and 2) are more likely to get filtered out by the spleen prematurely, leading to severe anemia. The effects of nitric oxide (NO) and its cogeners were rigorously tested and were shown to improve deformability under specific conditions.