Zaugg R H, Walder J A, Klotz I M
J Biol Chem. 1977 Dec 10;252(23):8542-8.
Normal and sickle erythrocytes were exposed in vitro to millimolar concentrations of 31 different carbonyl compounds. Schiff base (imine) linkages were formed with amino groups of intracellular hemoglobin. Adducts were isolated by gel electrofocusing and could be dissociated by dialysis. Aromatic aldehydes proved more reactive than aliphatic aldehydes, and ketones were unreactive. The influence of various ring substituents on the reactivity of aromatic aldehydes was found to conform closely to traditional concepts regarding electronic and steric effects. Several of the aromatic aldehydes were shown to markedly increase the oxygen affinity of hemoglobins A and S. In particular, 2,4-dihydroxybenzaldehyde and o-vanillin, at concentrations of 5 mM, produced 2- to 3-fold reductions in the P50 (partial pressure of oxygen at half-saturation) of sickle hemoglobin in whole blood. Since low degrees of oxygen saturation promote erythrocyte sickling, compounds of this type significantly inhibit sickling at reduced partial pressures of oxygen.
将正常红细胞和镰状红细胞在体外暴露于31种不同羰基化合物的毫摩尔浓度下。席夫碱（亚胺）键与细胞内血红蛋白的氨基形成。加合物通过凝胶电聚焦分离，并且可以通过透析解离。结果表明，芳香醛比脂肪醛更具反应性，而酮则无反应性。发现各种环取代基对芳香醛反应性的影响与关于电子效应和空间效应的传统概念密切相符。有几种芳香醛被证明能显著增加血红蛋白A和S的氧亲和力。特别是，2,4 - 二羟基苯甲醛和邻香草醛在浓度为5 mM时，使全血中镰状血红蛋白的P50（半饱和时的氧分压）降低了2至3倍。由于低程度的氧饱和度会促进红细胞镰变，这类化合物在降低的氧分压下能显著抑制镰变。
