Inhibition of erythrocyte sickling by cystamine, a thiol reagent.

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Abstract
Incubation of human sickle cells with cystamine, a thiol reagent, resulted in the formation of an intracellular S-ethylamine derivative. The rate of the reaction was dependent upon the cystamine concentration, the temperature and the duration of the incubation. The cystamine-treated cells demonstrated a marked inhibition of sickling under hypoxic conditions, a decrease in their mean corpuscular Hb concentration, and a significant increase in their O2 affinity. The O2 affinity of these cells was less dependent on their mean corpuscular Hb concentration than that of untreated sickle cells. The minimum gelling concentration of S-ethylamine deoxyhemoglobin S was slightly increased. Cystamine did not affect the intracellular pH nor the 2,3-diphosphoglycerate level. The exact contribution of the interrelated factors in cystamine inhibition of sickling (changes in O2 affinity, mean corpuscular Hb concentration and minimum gelling concentration) must be determined.