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    Hemoglobin. 1992;16(4):267-73.

    Hb Bab-Saadoun or alpha 2 beta (2)48(CD7)Leu----Pro, a mildly unstable variant found in an Arabian boy from Tunisia.

    Molchanova TP, Wilson JB, Gu LH, Guemira F, Fattoum S, Huisman TH.

    Source

    Department of Biochemistry and Molecular Biology, Medical College of Georgia, Augusta 30912-2100.

    Abstract

    Hb Bab-Saadoun which has a Leu----Pro substitution at position 48 of the beta chain was detected in a young Arabian boy living in Tunisia. His parents did not have the variant which suggests that it occurred as a spontaneous mutation. The substitution is located in the interhelical CD segment; leucine at beta 48 is an invariable amino acid that may be important as part of a spacer sequence between the two helices and its replacement by proline may affect the stability of the hemoglobin molecule. Hb Bab-Saadoun is unstable in heat and isopropanol stability tests and its chain was best isolated by parachloromercuribenzoate precipitation. It appears unlikely that the presence of Hb Bab-Saadoun results in a hemolytic anemia.

    PMID:
    1517103
    [PubMed - indexed for MEDLINE]

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