ANALYTICAL SUBCELLULAR FRACTIONATION OF NEUTROPHILS FROM PATIENTS WITH CHRONIC GRANULOMATOUS DISEASE - DEMONSTRATION OF ENZYME DEFECT IN 4 CASES

  • 1 January 1978
    • journal article
    • research article
    • Vol. 47  (186) , 213-220
Abstract
Analytical subcellular fractionation studies were performed on neutrophils from 5 patients, including 2 females, with chronic granulomatous disease. The density distribution and marker enzyme activities of the principal subecllular organelles in unstimulated cells were similar to those in unstimulated neutrophils from control subjects. NADH dependent reduction of nitro blue tetrazolium was measured in 4 of the patients including 1 female. In homogenates of whole cells the specific activity of this enzyme expressed as mU/mg protein was lower in the patients than in the controls, but the difference was not statistically signifcant. There was a highly significant difference between the specific activities of this enzyme in the plasma membrane fractions isolated from neutrophils of the 4 patients and the 3 controls. The primary microbicidal oxidase of neutrophils, defective function of which manifests as the syndrome of chronic granulomatous disease, is apparently a plasma membrane NADH oxidoreductase.