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Summary MATERIALS A N D METHODSCystic fibrosis (CF) has recently been linked to the group of human Three ml veinous blood were collected with heparin (62 Units diseases in which cultured fihrablasts express premature aging. As per 5 ml of whole blood) and immediately centrifuged (15 min, the deleterious effect of oxygen derivatives in the cell is onc of the 500 X g). Plasma and buffy coat were then removed and erythnumerous pathways associated with cell aging, the activity of the rocytes were washed twice in 0.15 M NaCI. The washed erythroenzymatic defcnse system, superoxide disrnutase (SOD), glutathi-cytes were then storcd at -70°C until assay. one peroxidase (GSHPx), glutathione reductase (GR), was exAll spectrophotometric determinations were performed in a amined in the erythrocytes of 12 CF children and waq compared Gilford recording spectrophotometer, model 240: GSHPx and GR to age-matched normal controls. No significant differences were at 37'C and SOD and GST at 25'C. SOD activity was measured found in CF children when compared to the controfs. Glutathione-in a readion system containing riboflavin and nitroblue tetrazo-S-transferase was also assayed, but the significant difference lium ( I l), with the following modification: before being eliminated found between CF chi1dren and normal contruIs is probably not by an ethanol-chloroform precipitation, hemoglobin is adjusted to specific of CF as it is found in other pathologic situations such as 8% in the hemolysate (16). In t h~s system, one unit corresponds to hyperhilirubinemia or renal insufficiency.0.5 pg of human copper-zinc SOD purified according to Hartz and Deutsch (9). GSRPx was measured by a coupled enzyme proceAbbreviations dure with glutathione reductase and NADPH using ter-butyl hydroperoxide as substrate (17). Hemoglobin was adjusted ta 2.5% CF, cystic fibrosisin the hemolysate and then converted to cyanmethemoglobin by FAD, flavin adenine dhucleotide potassium cyanide and potassium fesricyanide, in order to miai-GR, glutathione rductase mize its pudoperoxidase activity. One unit of GSBPx was taken GSHPx, glutat hione peroxidaseas the amount or enzyme that transformed 1 pmole of NADPH GST, glutathione-S-transferase under the assay conditions. Glutathione reductase was assayed SOD, superoxide dismutase according to Beutler (I), with and without the addition of exogenous FAD. One unit of enzyme activity was taken to be the amount of enzyme that t m s f o r m d 1 pmole of NADPH per min C*ic fibrosis (CF) is a lethal exocrinopathy transmitted as an under the assay conditions. Glutathione-S-transferase was assayed autosomal recessive condition, h which the abnormal gene defect with l-chlom-2,4-d~itrobenZe11e and GSH as substrates. The remains unknown despite numerous studis (5). It has been formation or the S-conjugate was monitored by following its ported that CF was part ofthat P U P of human diseases in which absorbance at 340 nm (2). One unit of enzyme activity is defined cultured fibroblatsexPTess premature aging (15 Table I. It has been repor...

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This paper studies three possible sites of action of drugs used for treatment of symptoms in ageing: blood (increasing flow, capillary bed, nutriment supply), the blood brain barrier (stabilization and/or increase of transfer rate), and the cell (increasing energetics, intermediary and/or neurotransmitter metabolism).

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