Hereditary myeloperoxidase deficiency

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Hereditary myeloperoxidase deficiency.

Subjects with neutrophil myeloperoxidase (MPO) deficiency have been rarely reported. In part this may be due to the lack of a simple screening technique that would detect them. With the routine use of a cytochemical leukocyte differential counter that employs MPO stains, over a 40-mo a period 8 unrelated probands with partial MPO-deficiency and one with complete deficiency were identified. Fami...

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Hereditary myeloperoxidase deficiency.

The functional properties of granulocytes in a diabetic patient deficient in myeloperoxidase (MPO) were compared with those of granulocytes in healthy subjects. The granulocytes of this patient had normal phagocytic activity. The microbicidal activity of the granulocytes was partially diminished with regard to Staphylococcus aureus and was almost nil with regard to Candida albicans. Fungicidal ...

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Biochemical and immunologic analysis of hereditary myeloperoxidase deficiency.

Myeloperoxidase (MPO), a heme enzyme present in the azurophilic granules of human polymorphonuclear neutrophils (PMN), is important in the oxygen-dependent microbicidal activity of PMN. MPO deficiency, defined as the lack of PMN peroxidative activity, is a common genetic defect of human PMN. The purpose of our study was to characterize the structural basis for this loss of enzymatic activity, u...

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Myeloperoxidase (MPO) gene mutation in hereditary MPO deficiency.

Myeloperoxidase (MPO), present in the azurophilic granules of polymorphonuclear leukocytes, is a myeloid enzyme whose synthesis is restricted to promyelocytes. Complete hereditary MPO deficiency affects 1 in 2,000 to 4,000 individuals; however, the genetic cause of this defect is unclear. We have determined the molecular basis of MPO deficiency in one individual (SQ). Granulocytes of SQ had no ...

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Leukocyte Myeloperoxidase Deficiency

This work was presented in part at the National Meeting of the American Federation for Clinical Research, Atlantic City, N. J., and published in abstract form (1). Received for publication 13 February 1969 antd in revised form 11 April 1969. Although lending support to the suggested bactericidal role of MPO in leukocytes, the data indicate that alternative bactericidal mechanisms, effective in ...

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ژورنال

عنوان ژورنال: Blood

سال: 1979

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood.v53.3.403.403