Hemoglobin New York [β113 (G 15) Val → Glu] Found in a Japanese Female with Cholelithiasis. Its Structure, Function, Instability and Biosynthesis

Abstract

In April 1982, during the course of hemoglobinopathy survey for the in-and out-patients of the Kinki University School of Medicine Hospital, Osaka Prefecture, we encountered an abnormal hemoglobin which migrated more slightly anodally to Hb A on isoelectric focusing (1)(Fig. 1). The carrier of the hemoglobin was a 43-year-old Japanese house-wife with cholelithiasis and chronic appendicitis, who was admitted to the hospital for surgical correction of these conditions. The operation disclosed an oval 1.7×2.5 cm combination gall stone incarcerated at the cholecystic duct, and it was removed. Hematological and chemical laboratory examinations of her peripheral blood shortly before the day of the surgical operation showed neither abnormalities nor hemolytic tendency (RBC 4. 96×10¹²/1, Hb 15.1 g/d1, PCV 0.436 1/1, MCV 87.7 fl, MCH 30.4 pg, MCHC 34.7 g/d1, reticulocyte count 1.3%, total bilirubin 0.8 mg/dl). This paper describes the results of studies on structure, function, instability and biosynthesis of this hemoglobin variant.