muramidase and Glucosephosphate-Dehydrogenase-Deficiency

Phagocytosis begins with exocytosis--"extrarapid" discharge of bactericidal proteins and factors of permeability into the extracellular medium. A viewpoint was put forward on an "avalanch-like" character of the outcome of cationic proteins from leukocyte granules in inflammation and their participation in formation of a nonphagocytic type of resistance. In phagocytosis bacteria perish due to the myeloperoxidase system, lysozyme, lactoferin and nonenzymic cationic proteins. Hereditary deficit of the above-mentioned substances leads to intraleukocytic microbicidal insufficiency, a drastic decrease in the nonspecific resistance of the organism and to development of fatal granulomatous disease, and to other forms of pathology associated with genetic defects of the bactericidal systems of leukocytes.

Topics: Blood Bactericidal Activity; Blood Proteins; Cell Membrane Permeability; Cytoplasmic Granules; Exocytosis; Glucosephosphate Dehydrogenase Deficiency; Histones; Humans; Immunity; Immunologic Deficiency Syndromes; Inflammation; Lactoferrin; Microscopy, Phase-Contrast; Muramidase; Neutrophils; Peroxidase; Phagocytosis

Topics: Agammaglobulinemia; Anemia, Sickle Cell; Chemotaxis; Female; Glucosephosphate Dehydrogenase Deficiency; Humans; Immunity, Cellular; Immunoglobulin E; Immunologic Deficiency Syndromes; Infections; Job Syndrome; Lactoferrin; Leukocytes; Male; Muramidase; Opsonin Proteins; Peroxidase; Phagocytes; Phagocytosis; Staphylococcus

Diseases of the blood and bone marrow are commonly associated with abnormalities of oxido-reductase and lysosomal enzymes within individual erythrocytes and leucocytes. There are considerable technical difficulties, however, in adapting enzyme histochemical techniques to the study of haemopoietic tissue since individual cells are readily disrupted during processing, show variable enzyme activity according to the stage of maturation, and possess a lipoprotein cytoplasmic membrane which hinders reagent penetration. Cytochemical techniques for the study of oxido-reductase systems are of importance in the study of the neutrophil in infected patients, the erythrocyte in glucose-6-phosphate dehydrogenase deficiency, and the primitive blast cell in acute leukaemia. Lysosomal enzymes are of importance in the study of the neutrophil in infected patients and in the differential diagnosis of acute leukaemia. Some examples of recent studies of these enzyme systems are given to illustrate technical procedures involving cytocentrifugation of cells on to glass slides, adjustment of the osmolality of the reaction mixture, and the study of smeared cells as opposed to cells incubated in suspension.

Topics: Acid Phosphatase; Alkaline Phosphatase; Aminopeptidases; Bone Marrow; Bone Marrow Cells; Erythrocytes; Esterases; Glucosephosphate Dehydrogenase Deficiency; Glucuronidase; Heterozygote; Histocytochemistry; Humans; Leukemia; Methods; Muramidase; NADH, NADPH Oxidoreductases; Neutrophils; Peroxidases; Sulfatases

Topics: Acid Phosphatase; Agranulocytosis; Blood Bactericidal Activity; Chediak-Higashi Syndrome; Chemotaxis; Child, Preschool; Complement System Proteins; Cytoplasmic Granules; Female; Glucosephosphate Dehydrogenase Deficiency; Humans; Immunologic Deficiency Syndromes; Infant, Newborn; Infant, Premature; Leukocytes; Liver; Lysosomes; Macrophages; Male; Monocytes; Mononuclear Phagocyte System; Muramidase; NADH, NADPH Oxidoreductases; Neutrophils; Opsonin Proteins; Peroxidases; Phagocyte Bactericidal Dysfunction; Phagocytosis; Spleen

Topics: Blood Glucose; Carbohydrate Metabolism, Inborn Errors; Carbohydrates; Citric Acid Cycle; Clinical Enzyme Tests; DNA; Down Syndrome; Galactosemias; Glucosephosphate Dehydrogenase Deficiency; Glycogen; Glycogen Storage Disease; Glycolysis; Glycosaminoglycans; Humans; Leukemia; Leukocytes; Muramidase; Neutrophils; Pentosephosphates; Phagocytosis; Propionates; RNA