title: A monoclonal antibody-linked immunoassay for hemoglobin H disease.
abstract: A murine monoclonal antibody (mAb) was generated that recognizes hemoglobin (Hb) H, the tetrameric form (beta 4) of human beta-globin chains. The antibody beta 4-1 (gamma 1, kappa) does not react with Hbs A, F, Bart's, or isolated beta chains, indicating that the antibody recognizes an epitope comprised of multiple beta chains. A simple, rapid, and sensitive enzyme immunoassay was established to detect and quantitate Hb H in hemolysates from subjects with Hb H disease. The delta globin level in these patients was also measured using the monoclonal antibody delta-1, which is specific for delta chains of Hb A2. With these assays, 20 hemolysates from subjects with Hb H disease' ten from normal adults and ten from newborn babies were analyzed. The percent of Hb H ranged from 1.5% to 25% in Hb H patients. There was a significant average reduction (32%) in delta chains in these samples as compared with the normal average adult value. The decreased expression of alpha chains thus results in a reduction of the levels of normal Hbs A and A2 and accumulation of beta 4, causing Hb H disease.
authors.affiliation: Department of Immunology and Microbiology, Medical College of Georgia, Augusta 30912-2400.
authors.forename: M
authors.initials: M
authors.lastname: Shyamala
authors.forename: C R
authors.initials: CR
authors.lastname: Kiefer
authors.forename: H
authors.initials: H
authors.lastname: Moscoso
authors.forename: F A
authors.initials: FA
authors.lastname: Garver
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods*; D006447:Hemoglobin H / Q000032:analysis / Q000276:immunology; D006801:Humans; D007231:Infant, Newborn; D012680:Sensitivity and Specificity; D013789:Thalassemia / Q000175:diagnosis*
chemical_list: D000911:Antibodies, Monoclonal; D006447:Hemoglobin H