Hemoglobin Hofu or αβ [126 (H4) Val → Glu] Found in Combination with Hemoglobin S

G. Brittenham Divisions of Hematology and Geographic Medicine, Departments of Medicine, Pediatrics Case Western Reserve University School of Medicine, Cleveland, Ohio, 44106, USA

B. Lozoff Department of Medicine Rangaraya, Medical College Kakinada, Andra Pradesh India, 533001

J. W. Harris Laboratory of Protein Chemistry and Comprehensive Sickle Cell Center Department of Cell, Molecular Biology, Medical College of Georgia, Augusta, Georgia, 30901, USA

N. V. S. Nayudu Department of Medicine Rangaraya, Medical College Kakinada, Andra Pradesh India, 533001

M. Gravely Laboratory of Protein Chemistry and Comprehensive Sickle Cell Center Department of Cell, Molecular Biology, Medical College of Georgia, Augusta, Georgia, 30901, USA

J. B. Wilson Laboratory of Protein Chemistry and Comprehensive Sickle Cell Center Department of Cell, Molecular Biology, Medical College of Georgia, Augusta, Georgia, 30901, USA

H. Lam Laboratory of Protein Chemistry and Comprehensive Sickle Cell Center Department of Cell, Molecular Biology, Medical College of Georgia, Augusta, Georgia, 30901, USA

T. H. J. Huisman Laboratory of Protein Chemistry and Comprehensive Sickle Cell Center Department of Cell, Molecular Biology, Medical College of Georgia, Augusta, Georgia, 30901, USA

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Abstract

Hb Hofu, αβ[126 (H4) Val → Glu], was found in 10 members of 2 apparently unrelated Valmiki families in central India. None showed evidence of hemolysis and hemoglobin levels were normal in most. In two individuals, Hb Hofu occurred in combination with Hb S, but neither had clinical manifestations of sickle cell disease. In samples containing Hb Hofu, the isopropanol precipitation test was positive. Quantitation of the hemoglobin fractions by DEAE-cellulose chromatography showed that Hb Hofu constituted a mean of 23-25% of the total whether in combination with Hb A or Hb S.

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Hemoglobin Hofu or αβ [126 (H4) Val → Glu] Found in Combination with Hemoglobin S

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Hemoglobin Hofu or αβ [126 (H4) Val → Glu] Found in Combination with Hemoglobin S

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