Valency hybrids of hemoglobin in red cells of patients with hereditary enzymopenic methemoglobinemia.

Twenty eight patients with hereditary methemoglobinemia have been found and examined. Using the techniques of analytic isoelectrofucossing in polyacrylamide gel and the preparative isoelectrofocussing on a column in sucrose gradient we have managed to disclose an abnormal fraction in hemolysate of the above mentioned patients. It has been proved that the obtained fraction contains hemoglobin valent hybrids, possesses high affinity to oxygen and low cooperativity. Due to the presence of valency hybrids about one-half of the quantity of oxy-Hb fails to participate in the normal function of oxygen delivery, which evidently is the molecular cause of disturbance of oxygen delivery in patients with hereditary enzymopenic methemoglobinemia. This also explains a high degree of cyanosis in them.