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Research Article

ScienceAsia 22 (1996): 117-120 |doi: 10.2306/scienceasia1513-1874.1996.22.117

PRESENCE OF cs-GLOBIN ON MEMBRANE OF RED CELLS CONTAINING HEMOGLOBIN CONSTANT SPRING (CS)

CHAYANON PEERAPITIAYAMONGKOLa, LUIGI F. BERNINIb AND PRAPON WILAIRATa

ABSTRACT: Hemoglobin (Hb) Constant Spring (CS) is an elongated -globin variant present at polymorphic frequencies in Southeast Asian countries. Analysis of membrane from red blood cells of individuals with Hb H/CS and Hb CS/CS by sodium dodecyl sulfate polyacrylamide gel-electrophoresis revealed the presence of a protein band with molecular size slightly greater than globin. This band reacted with rabbit anti-human -globin antibodies in a Western blot assay. The identity of this membrane-bound -globin-like protein as being CS-globin was verified by comparing its ratio of biosynthetically [3H]-labelled proline[14C]-labelled lysine with those of normal globin chains. The presence of CS-globin on the membrane of Hb H/CS and Hb CS/CS red cells affords an explanation for the unusually severe anemia observed in these subjects.

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a Department of Biochemistry, Faculty of Science, Mahidol University, Rama VI Road, Bangkok 10400, Thailand.
b Department of Human Genetics, Medical Genetic Center, Leiden State University, 2333 AL, Leiden, The Netherlands

Received April 25, 1996