Nitrogen mustard and nor-nitrogen mustard inhibit sickling, but the concentrations required would be associated with unacceptable toxicity if these agents were administered to patients. Red cells could be treated extracorporeally and infused back into donors, if the alkylating agent could be removed or inactivated, if the treatment per se did not significantly shorten red cell survival, and if ...

Cyanate reacts with the amino-terminal valine residues of hemoglobin S and prevents the sickling in vitro of 50-80% of the erythrocytes from patients with sickle-cell disease. The purpose of the studies reported here was to determine whether this anti-sickling effect would increase the survival of cyanate-treated cells that were returned to the patient. In seven subjects with sickle-cell diseas...

V. NEEL, M.D., PH.D. T HIS REPORT deals with a new inherited abnormality of hemoglobin encountered in American Negroes. The existence of this traitcame to light ill the course of extensive clinical and genetic studies u’egarding the sickling pheuiomenon. Among the individuals examined thei’e were anumber of patients ‘it-h a hemolytic syndrome associated with erythrocytic sickling in whom neithe...

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...

Incubation of sickle cells with cystamine, a thiol reagnet, 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 hemo...