title: Myotonic dystrophy: defective oxidative burst of polymorphonuclear leukocytes.
abstract: Because myotonic dystrophy (MD) is an autosomal dominant multisystemic disorder affecting plasma membrane, we have studied the oxidative burst of PMNs. The PMA and fMet-Leu-Phe-stimulated superoxide generation is defective in the patient group as compared to controls: the response is both delayed and low. The kinetic parameters of the NADPH oxidase complex are not affected. We have not found any abnormalities in the membrane potential changes. In addition, the cytosolic protein kinase C (PKC) activity of resting PMNs is similar in MD patients and controls, and the translocation of protein kinase C in response to PMA is not impaired. The decrease of the oxidative response of PMNs from MD patients may be related to an abnormality of the environment of the NADPH oxidase.
authors.affiliation: Lab. Immunology, Hopital Sainte Marguerite, Marseille, France.
authors.forename: J L
authors.initials: JL
authors.lastname: Mege
authors.forename: J
authors.initials: J
authors.lastname: Pouget
authors.forename: C
authors.initials: C
authors.lastname: Capo
authors.forename: P
authors.initials: P
authors.lastname: Andre
authors.forename: A M
authors.initials: AM
authors.lastname: Benoliel
authors.forename: G
authors.initials: G
authors.lastname: Serratrice
authors.forename: P
authors.initials: P
authors.lastname: Bongrand
mesh_terms: D000328:Adult; D006801:Humans; D008564:Membrane Potentials; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000378:metabolism*; D009240:N-Formylmethionine Leucyl-Phenylalanine / Q000494:pharmacology; D009247:NADH, NADPH Oxidoreductases / Q000032:analysis; D019255:NADPH Oxidases; D009504:Neutrophils / Q000378:metabolism*; D010084:Oxidation-Reduction; D011493:Protein Kinase C / Q000032:analysis; D013481:Superoxides / Q000378:metabolism*; D013755:Tetradecanoylphorbol Acetate / Q000494:pharmacology
chemical_list: D013481:Superoxides; D009240:N-Formylmethionine Leucyl-Phenylalanine; D009247:NADH, NADPH Oxidoreductases; D019255:NADPH Oxidases; D011493:Protein Kinase C; D013755:Tetradecanoylphorbol Acetate