Quantitative analysis of abnormal hemoglobins by agar gel electrophoresis. 1985

I Steiman, and C Norwood, and S Charache

Measurement of the proportion of abnormal hemoglobin in a hemolysate is essential for differentiation of simple and compound heterozygotes (e.g., sickle cell trait vs. sickle thalassemia), for differential of alpha- and beta-thalassemia in compound heterozygotes, and for differentiation of various types of beta-thalassemia in such persons. Utilization of such measurements is hampered by the imprecision and inconvenience of current methods. We have adapted a readily available agar electrophoresis method for this purpose, scanning unstained gels at 420 nm. The new method is particularly valuable for rapid estimation of percent of HbS after partial exchange transfusion in patients with sickle cell anemia. It cannot be used for quantitation of HbF or for quantitation of hemoglobins that comigrate with HbA; contrariwise, it can be used for hemoglobins that only separate from HbA on agar (e.g., HbBethesda).

UI MeSH Term Description Entries
D001797 Blood Protein Electrophoresis Electrophoresis applied to BLOOD PROTEINS. Hemoglobin Electrophoresis,Electrophoresis, Blood Protein,Electrophoresis, Hemoglobin,Protein Electrophoresis, Blood
D004587 Electrophoresis, Agar Gel Electrophoresis in which agar or agarose gel is used as the diffusion medium. Electrophoresis, Agarose Gel,Agar Gel Electrophoresis,Agarose Gel Electrophoresis,Gel Electrophoresis, Agar,Gel Electrophoresis, Agarose
D006445 Hemoglobin C Disease A disease characterized by compensated hemolysis with a normal hemoglobin level or a mild to moderate anemia. There may be intermittent abdominal discomfort, splenomegaly, and slight jaundice. Hemoglobin-C Disease,C Disease, Hemoglobin,C Diseases, Hemoglobin,Hemoglobin C Diseases,Hemoglobin-C Diseases
D006451 Hemoglobin, Sickle An abnormal hemoglobin resulting from the substitution of valine for glutamic acid at position 6 of the beta chain of the globin moiety. The heterozygous state results in sickle cell trait, the homozygous in sickle cell anemia. Hemoglobin S,Deoxygenated Sickle Hemoglobin,Deoxyhemoglobin S,Hemoglobin SS,Hemoglobin, Deoxygenated Sickle,SS, Hemoglobin,Sickle Hemoglobin,Sickle Hemoglobin, Deoxygenated
D006455 Hemoglobins, Abnormal Hemoglobins characterized by structural alterations within the molecule. The alteration can be either absence, addition or substitution of one or more amino acids in the globin part of the molecule at selected positions in the polypeptide chains. Abnormal Hemoglobins
D006461 Hemolysis The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity. Haemolysis,Extravascular Hemolysis,Intravascular Hemolysis,Extravascular Hemolyses,Haemolyses,Hemolyses, Extravascular,Hemolyses, Intravascular,Hemolysis, Extravascular,Hemolysis, Intravascular,Intravascular Hemolyses
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000755 Anemia, Sickle Cell A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S. Hemoglobin S Disease,HbS Disease,Sickle Cell Anemia,Sickle Cell Disease,Sickle Cell Disorders,Sickling Disorder Due to Hemoglobin S,Anemias, Sickle Cell,Cell Disease, Sickle,Cell Diseases, Sickle,Cell Disorder, Sickle,Cell Disorders, Sickle,Disease, Hemoglobin S,Hemoglobin S Diseases,Sickle Cell Anemias,Sickle Cell Diseases,Sickle Cell Disorder
D012805 Sickle Cell Trait The condition of being heterozygous for hemoglobin S. Cell Trait, Sickle,Cell Traits, Sickle,Sickle Cell Traits,Trait, Sickle Cell,Traits, Sickle Cell
D013789 Thalassemia A group of hereditary hemolytic anemias in which there is decreased synthesis of one or more hemoglobin polypeptide chains. There are several genetic types with clinical pictures ranging from barely detectable hematologic abnormality to severe and fatal anemia. Thalassemias

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