Progressive muscular dystrophy (Duchenne): biochemical studies by flow-cytometry.
The peanut lectin (PNL) receptor density of the cell membrane and several metabolic parameters of cultured fibroblasts of normal human individuals and of patients with muscular dystrophy were measured by simultaneous two and three parameter flow cytometry. The PNL-receptor density was significantly decreased on muscular dystrophy fibroblasts (between 20.7 and 33.6%) as compared to normal fibroblasts. The cell volume, the esterase activity, the intracellular pH, and the percentage of proliferating cells of both types of fibroblasts were not significantly altered. The mean cell volume of different fibroblast cultures varied between 2500 and 6000 micron 3. The concentration of the intracellular esterase activity of fibroblasts was low (0.169 relative units) as compared to lymphocytes and granulocytes of the peripheral blood (1.56 and 2.17 relative units). The fibroblasts had an acidic intracellular pH of 6.52 while lymphocytes and granulocytes had basic pH values of 7.30 and 7.17. Some of the fibroblasts were in the S + G2/M phase of the cell cycle (20%). The study shows that the measurement of biochemical parameters of vital and fixed single fibroblasts by flow-cytometry is of great interest for the recognition of differences between normal individuals and muscular dystrophy patients.
D002453:Cell Cycle; D002470:Cell Survival; D002478:Cells, Cultured; D002648:Child; D002675:Child, Preschool; D004950:Esterases / Q000378:metabolism*; D005260:Female; D005347:Fibroblasts / Q000378:metabolism / Q000473:pathology; D005434:Flow Cytometry; D006651:Histocytochemistry; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008297:Male; D009136:Muscular Dystrophies / Q000378:metabolism* / Q000473:pathology; D011975:Receptors, Mitogen / Q000378:metabolism*
D011975:Receptors, Mitogen; C038223:peanut agglutinin receptor; D004950:Esterases