[Peroxidase activity of neutrophil granules in two cases of congenital myeloperoxidase deficiency].
The neutrophils and monocytes of two patients with hereditary myeloperoxidase deficiency lacked myeloperoxidase activity as determined by light and electron microscopic cytochemical staining. Using Graham-Karnovsky media, azurophils of neutrophils were devoid of peroxidase whereas all eosinophilic and basophilic granules exhibited normal peroxidase activity. After incubation in alkaline diaminobenzidine media which stains the catalase of microperoxisomes, some small granules were seen to be strongly stained in both immature and mature neutrophils. These small granules were distinct from all other neutrophilic granules which lacked a positive reaction. Only, in the presence of cyanide or aminotriazole, peroxidatic activity was also detected in some ellipsoid azurophils. This observation suggests that these substances activated an oxidase whose nature is discussed.
D001491:Basophils / Q000201:enzymology; D002374:Catalase / Q000097:blood; D003486:Cyanides / Q000494:pharmacology; D003594:Cytoplasmic Granules / Q000201:enzymology; D004804:Eosinophils / Q000201:enzymology; D006651:Histocytochemistry; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008661:Metabolism, Inborn Errors / Q000097:blood; D008830:Microbodies / Q000201:enzymology; D008854:Microscopy, Electron; D009000:Monocytes / Q000201:enzymology / Q000648:ultrastructure; D009504:Neutrophils / Q000201:enzymology* / Q000648:ultrastructure; D009195:Peroxidase / Q000172:deficiency*; D010544:Peroxidases / Q000037:antagonists & inhibitors / Q000097:blood / Q000172:deficiency*
D003486:Cyanides; D010544:Peroxidases; D002374:Catalase; D009195:Peroxidase
Activités peroxydasiques de certaines granulations des neutrophiles dans deux cas de déficit congénital en myéloperoxydase