Abstract

Plasma chondroitin sulfate (CS) amount and charge density were determined in 45 healthy volunteers (control group), 45 pseudoxanthoma elasticum (PXE)-affected patients and 19 healthy carriers by using fluorophore-assisted carbohydrate electrophoresis (FACE) and HPLC equipped with postcolumn derivatization and fluorescence detection. The mean values of CS amount were 4.9 ± 1.21 for volunteers, 4.7 ± 1.40 for PXE subjects and 4.4 ± 1.44 for the carriers. No significant differences were found for the three human subjects groups. On the contrary, by considering the age of normal volunteers, a significant increase of plasma CS amount was measured. In fact, the volunteers aging from 17 to 40 years (mean 32.1) showed a CS concentration of 4.3 ± 1.30 while the group ranging from 50 to 74 years (mean 56.9) had a value of 5.6 ± 1.16 with a significant increase of + 30.2%. The same significant increase in CS plasma content with increasing age was measured for PXE-affected and healthy carriers group. Extracted plasma CS was evaluated for the main two unsaturated disaccharides, non-sulfated and 4-monosulfated, and the charge density determined. The mean values were 0.54 ± 0.13 for volunteers, 0.60 ± 0.15 for PXE subjects and 0.50 ± 0.15 for the carriers. A significant increase of + 11.1% was found between the PXE patients and healthy human group but no differences were calculated between the control group and the carriers. Furthermore, besides a CS amount, the volunteers aging from 17 to 40 years (mean 32.1) showed a charge density of 0.53 ± 0.14 while the group ranging from 50 to 74 years (mean 56.9) had a value of 0.58 ± 0.17 with a significant increase of + 9.4%. The same trend was measured for the healthy carriers group. The CS charge density of PXE-affected subjects was found to increase significantly more than healthy controls depending on the age. In fact, the PXE patients aging from 10 to 40 years (mean 29.3) showed a charge density of 0.56 ± 0.14 while the group ranging from 50 to 74 years (mean 58.6) had a value of 0.67 ± 0.11 with a significant increase of + 19.6%. Furthermore, the group of PXE-affected subjects ranging from 50 to 74 years (mean 58.6) showed a significant increase of 15.5% in comparison with the group matched for age (mean 56.9) of healthy volunteers.

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