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- W2093442723 abstract "A more sensitive assay procedure has been developed for the enzyme iduronate 2-sulphate sulphatase which is deficient in the Hunter syndrome. The substrate is the same as previously described by Lim et al. [1], O-(α-l-idopyranosyluronic acid 2-sulphate)-(1 → 4)-2,5 anhydro-d-[3H-1]mannitol 6-sulphate, but, after incubation, it is separated from the product by ion-exchange chromatography on a micro-column of Dowex 1 × 2 (Cl−) instead of high voltage electrophoresis or ECTEOLA cellulose chromatography. Since the blank correction is then much smaller, a shorter incubation time can be used and conversion of the substrate reduced from approximately 50% down to levels where complications resulting from substrate depletion and product inhibition are minimal. Using whole serum the apparent Km for the substrate is 0.2 mmol/1. With an incubation time of 20 min, sera from heterozygotes exhibited approximately 35% of the normal levels of iduronate 2-sulphate sulphatase (0.11–0.61, mean 0.34 nmol.h−1.mg−1 protein for 21 carriers; 0.24–2.35, mean 0.94 nmol.h−1.mg−1 protein for 37 normal females). Serum analyses can thus be used to supplement those on hair roots in the detection of carriers of the Hunter syndrome." @default.
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- W2093442723 date "1981-04-01" @default.
- W2093442723 modified "2023-09-26" @default.
- W2093442723 title "An improved assay for iduronate 2-sulphate sulphatase in serum and its use in the detection of carriers of the Hunter syndrome" @default.
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- W2093442723 doi "https://doi.org/10.1016/0009-8981(81)90274-6" @default.
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