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Characterization of a variant prothrombin in a patient congenitally deficient in factors II, VII, IX and X.

Abstract

An 18-month-old child, who had no evidence of liver disease, malabsorption, or chronic ingestion of coumarin compounds, was found to have plasma deficiencies of factors II, VII, IX and X. Assays for factor II and X by immunological techniques (antibody neutralization and immunoelectrophoresis) revealed normal or elevated antigenic activity of these factors, suggesting the presence of abnormal protein variants in the patient's plasma. On two-dimensional immunoelectrophoresis of the patient's plasma in calcium, a normal and an abnormal population of prothrombin were seen. The abnormal prothrombin had a mobility more anodal than that of normal prothrombin, but less anodal than that of acarboxyprothrombin. The abnormal prothrombin, in contrast to acarboxyprothrombin, adsorbed readily to both aluminum hydroxide and barium citrate, and could be identified by two-dimensional immunoelectrophoresis of a barium citrate eluate. We suspect that the abnormal variant represents a partially carboxylated prothrombin.

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  • Publisher Full Text
  • Authors

    , , , ,

    Source

    British journal of haematology 44:3 1980 Mar pg 461-9

    MeSH

    Blood Coagulation Disorders
    Calcium
    Factor VII Deficiency
    Factor X Deficiency
    Hemophilia B
    Humans
    Hypoprothrombinemias
    Immunoelectrophoresis, Two-Dimensional
    Infant
    Male
    Protein Binding
    Prothrombin

    Pub Type(s)

    Case Reports
    Journal Article
    Research Support, U.S. Gov't, P.H.S.

    Language

    eng

    PubMed ID

    7378310

    Citation

    Johnson, C A., et al. "Characterization of a Variant Prothrombin in a Patient Congenitally Deficient in Factors II, VII, IX and X." British Journal of Haematology, vol. 44, no. 3, 1980, pp. 461-9.
    Johnson CA, Chung KS, McGrath KM, et al. Characterization of a variant prothrombin in a patient congenitally deficient in factors II, VII, IX and X. Br J Haematol. 1980;44(3):461-9.
    Johnson, C. A., Chung, K. S., McGrath, K. M., Bean, P. E., & Roberts, H. R. (1980). Characterization of a variant prothrombin in a patient congenitally deficient in factors II, VII, IX and X. British Journal of Haematology, 44(3), pp. 461-9.
    Johnson CA, et al. Characterization of a Variant Prothrombin in a Patient Congenitally Deficient in Factors II, VII, IX and X. Br J Haematol. 1980;44(3):461-9. PubMed PMID: 7378310.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Characterization of a variant prothrombin in a patient congenitally deficient in factors II, VII, IX and X. AU - Johnson,C A, AU - Chung,K S, AU - McGrath,K M, AU - Bean,P E, AU - Roberts,H R, PY - 1980/3/1/pubmed PY - 1980/3/1/medline PY - 1980/3/1/entrez SP - 461 EP - 9 JF - British journal of haematology JO - Br. J. Haematol. VL - 44 IS - 3 N2 - An 18-month-old child, who had no evidence of liver disease, malabsorption, or chronic ingestion of coumarin compounds, was found to have plasma deficiencies of factors II, VII, IX and X. Assays for factor II and X by immunological techniques (antibody neutralization and immunoelectrophoresis) revealed normal or elevated antigenic activity of these factors, suggesting the presence of abnormal protein variants in the patient's plasma. On two-dimensional immunoelectrophoresis of the patient's plasma in calcium, a normal and an abnormal population of prothrombin were seen. The abnormal prothrombin had a mobility more anodal than that of normal prothrombin, but less anodal than that of acarboxyprothrombin. The abnormal prothrombin, in contrast to acarboxyprothrombin, adsorbed readily to both aluminum hydroxide and barium citrate, and could be identified by two-dimensional immunoelectrophoresis of a barium citrate eluate. We suspect that the abnormal variant represents a partially carboxylated prothrombin. SN - 0007-1048 UR - https://www.unboundmedicine.com/medline/citation/7378310/Characterization_of_a_variant_prothrombin_in_a_patient_congenitally_deficient_in_factors_II_VII_IX_and_X_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0007-1048&date=1980&volume=44&issue=3&spage=461 DB - PRIME DP - Unbound Medicine ER -