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Myotonic Dystrophy: Defective Oxidative Burst of Polymorphonuclear Leukocytes
Author(s) -
Mege J.L.,
Pouget J.,
Capo C.,
Andre P.,
Benoliel A.M.,
Serratrice G.,
Bongrand P.
Publication year - 1988
Publication title -
journal of leukocyte biology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.819
H-Index - 191
eISSN - 1938-3673
pISSN - 0741-5400
DOI - 10.1002/jlb.44.3.180
Subject(s) - respiratory burst , nadph oxidase , superoxide , biology , myotonic dystrophy , oxidative phosphorylation , protein kinase c , superoxide dismutase , oxidative stress , oxidase test , kinase , biochemistry , microbiology and biotechnology , immunology , medicine , endocrinology , enzyme , genetics
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.