CHARACTERIZATION OF HEREDITARY PARTIAL MYELOPEROXIDASE DEFICIENCY

  • 1 January 1982
    • journal article
    • research article
    • Vol. 99  (4) , 589-600
Abstract
A family with a partial myeloperoxidase deficiency was studied. The myeloperoxidase in the neutrophils and monocytes of the parents and their 2 sons had normal spectral properties (determined optically and by EPR). Enzymic characteristics (oxidation of iodide) were indistinguishable from those of normal myeloperoxidase; immunological indentity between the myeloperoxidase in the leukocytes of the family members and normal myeloperoxidase was found. No differences in heat stability were observed. The neutrophils and monocytes of the sons contained 9-18% of the myeloperoxidase content of normal cells; the neutrophils and monocytes of the parents contained 45-58%. Apparently the parents are heterozygous and the sons homozygous for hereditary partial myeloperoxidase deficiency or each parent is heterozygous for a different type of myeloperoxidase deficiency and the sons combine both deficiencies. The oxidative metabolism of the neutrophils during phagocytosis was not affected by the myeloperoxidase deficiency. The killing of Staphylococcus aureus was apparently normal. The perforation of Escherichia coli by the neutrophils of the sons was retarded in comparison with normal neutrophils.