Valency hybrids of hemoglobin in red cells of patients with hereditary enzymopenic methemoglobinemia. 1977

A P Andreyeva, and M G Dmitriyeva, and A A Levina, and L M Tsibulskaya, and Y G Kazanetz, and I I Ilyinskaya, and I V Derviz, and Y N Tokarev

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.

UI MeSH Term Description Entries
D007525 Isoelectric Focusing Electrophoresis in which a pH gradient is established in a gel medium and proteins migrate until they reach the site (or focus) at which the pH is equal to their isoelectric point. Electrofocusing,Focusing, Isoelectric
D008706 Methemoglobin Ferrihemoglobin
D008708 Methemoglobinemia The presence of methemoglobin in the blood, resulting in cyanosis. A small amount of methemoglobin is present in the blood normally, but injury or toxic agents convert a larger proportion of hemoglobin into methemoglobin, which does not function reversibly as an oxygen carrier. Methemoglobinemia may be due to a defect in the enzyme NADH methemoglobin reductase (an autosomal recessive trait) or to an abnormality in hemoglobin M (an autosomal dominant trait). (Dorland, 27th ed) Methemoglobinemias
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010100 Oxygen An element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration. Dioxygen,Oxygen-16,Oxygen 16
D010108 Oxyhemoglobins A compound formed by the combination of hemoglobin and oxygen. It is a complex in which the oxygen is bound directly to the iron without causing a change from the ferrous to the ferric state. Oxycobalt Hemoglobin,Oxycobalthemoglobin,Oxyhemoglobin,Hemoglobin, Oxycobalt
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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