Comparison of cetylpyridinium chloride and cetyltrimethylammonium bromide extractive procedures for quantification and characterization of human urinary glycosaminoglycans

Buzzega, Dania; Pederzoli, Francesca; Maccari, Francesca; Aslan, Diler; Türk, Mehmet Sezai; Volpi, Nicola

doi:10.1515/cclm.2010.217

Cited by 6 publications

(1 citation statement)

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“…Urine GAGs collected before ERT and at different days and times during therapy over a period of 10 months were extracted by means of a conventional method (Dietrich et al 1993;Maccari et al 2003;Buzzega et al 2010) and The results are the mean of three different analyses. The coefficient of variation % was always found to be lower than 15% for all analyses.…”

Section: Urine Gag Compositionmentioning

confidence: 99%

Plasmatic and Urinary Glycosaminoglycans Characterization in Mucopolysaccharidosis II Patient Treated with Enzyme-Replacement Therapy with Idursulfase

et al. 2011

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We report the structural characterization of plasmatic and urinary GAGs in a patient affected by MPS II (Hunter syndrome) before and during the first 10 months of enzyme-replacement therapy (ERT). Plasmatic GAGs before ERT were rich in pathological DS consisting of iduronic acid (IdoA) and composed of ~90% ΔDi4s and trace amounts of disulfated disaccharides. DS was also characterized as the main (~90%) urinary GAG mainly composed of ~90% ΔDi4s with minor percentages of monosulfated and disulfated disaccharides, in particular ΔDi2,4dis. After 300 days of ERT, plasmatic DS strongly decreased but ~14% of IdoA-rich ΔDi4s was still detected. Similarly, urinary galactosaminoglycans were mainly composed of 78% ΔDi4s, ~11% ΔDi6s and ~4% ΔDi0s with the persistence of ΔDi2,4dis (~4%). About 40% of IdoA-formed ΔDi4s were also calculated, thus confirming that pathological DS is still present in excreted urinary GAGs during ERT. By considering the % of IdoA, we observed rather similar kinetics of excretion in fluids from the beginning of the treatment. Immediately after the first enzyme infusion, a large amount of abnormal DS is removed from tissues reaching the blood compartment and eliminated via the urine, and this process lasts for about 2 weeks. After this, the percentage of IdoA-rich material present in biological fluids remains fairly constant over the following 9 months of treatment. To date, these are the first data regarding plasmatic and urinary kinetics directly measured on products released by the activity of the recombinant enzyme Idursulfase, iduronate-2-sulfatase, evaluated using specific and sensitive analytical procedures.

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Section: Urine Gag Compositionmentioning

confidence: 99%