Increased monocyte oxidase activity in cystic fibrosis heterozygotes and homozygotes.
Freshly isolated monocytes from cystic fibrosis (CF) heterozygotes and homozygotes had significantly increased oxygen uptake and superoxide formation after surface glycoprotein stimulation than did monocytes from age- and sex-matched controls. Lack of differences among the genotypes in inhibition by simple sugars of the concanavalin A-stimulated superoxide production and lack of differences in concanavalin A-binding surface proteins suggested that different regulation of the oxidase pathway produced the increased oxygen uptake and superoxide formation in CF patients and carriers. This regulatory role is consistent with the predicted structure of the CF gene product. The results support the hypothesis that the mononuclear phagocytes of CF heterozygotes have a significantly increased ability to kill intracellular microbes and may confer a selective advantage to the host.
Regelmann
W E
WE
Department of Pediatrics, University of Minnesota, Minneapolis.
Skubitz
K M
KM
Herron
J M
JM
D000293:Adolescent; D000328:Adult; D002648:Child; D003208:Concanavalin A / Q000494:pharmacology; D003550:Cystic Fibrosis / Q000097:blood / Q000201:enzymology* / Q000235:genetics; D003576:Electron Transport Complex IV / Q000037:antagonists & inhibitors; D005260:Female; D006580:Genetic Carrier Screening; D005838:Genotype; D006720:Homozygote; D006801:Humans; D008163:Luminescent Measurements; D008297:Male; D008565:Membrane Proteins / Q000097:blood; D009000:Monocytes / Q000187:drug effects / Q000201:enzymology* / Q000378:metabolism; D010088:Oxidoreductases / Q000097:blood*; D010101:Oxygen Consumption; D010544:Peroxidases / Q000097:blood
D008565:Membrane Proteins; D003208:Concanavalin A; D010088:Oxidoreductases; D010544:Peroxidases; D003576:Electron Transport Complex IV