how to cite this article: abdi m, khatami sh, hakhamaneshi ms, alaei mr, azadi na, zamanfar d, taghikhani m.validation of urinary glycosaminiglycans in iranian patients with mucopolysaccharidose type i: the effect of urine sedimentation characteristics. iran j child neurol. 2014; 8(4):39-45.   abstract objective the first line-screening test for mucopolysaccharidosis is based on measurement of ...

A microsomal preparation from chick embryo cartilage has been shown to catalyze the incorporation of sulfate-36S from 3’-phosphoadenosine 5’-phosphosulfate-3% into microsomal glycosaminoglycan. This enzyme preparation is the same preparation that previously has been shown to catalyze the formation of chondroitin (nonsulfated) from sugar nucleotide precursors. The microsomal sulfate acceptor app...

sulphated glycosaminoglycan content: preliminary results of an in-vivo validation study using an ex-vivo reference standard for cartilage sulphated glycosaminoglycan content Jasper van Tiel, Gyula Kotek, Max Reijman, Pieter K. Bos, Esther E. Bron, Stefan Klein, Jan A. Verhaar, Gabriel P. Krestin, Harrie Weinans, and Edwin H. Oei Radiology, Erasmus MC, University Medical Center Rotterdam, Rotter...

A capillary electrophoresis method was developed for the analysis of oligosaccharides combined with derivatization with 2-aminobenzoic acid. Glycosaminoglycan delta-disaccharides were effectively resolved on a fused-silica capillary tube using 150 mM borate, pH 8.5, as a running electrolyte solution. This analytical method was applied to the identification of glycosaminoglycan in combination wi...

Capillary electrophoresis is a common technique used for glycosaminoglycan-derived disaccharide analysis because of its high resolving power, high separation efficiency, high sensitivity, short analysis time, and straightforward operation. CE coupled to laser-induced fluorescence (LIF) detection shows an approximately 100 times higher sensitivity than traditional UV detection at 232 nm. 2-Amino...

Glycosaminoglycan production, acid hydrolase activity, proliferation, and morphology were examined in human synovial cells subjected to low environmental pH. The amount and the molecular size of newly synthesised glycosaminoglycan (GAG) were increased without significant change in the rate of cell proliferation. Lowered pH produced an increase in the size of cytoplasmic organelles. Some of thes...

Specific sensory input to neocortex arrives in layer 4, whereas nonspecific input, such as information about salience or novelty, is thought to arrive in layer 1. This scheme might imply a slower arrival of inputs to layer 1. In this issue, Zhu and Zhu examine this question using paired whole-cell recording in vivo from layer 1 nonpyramidal cells and the dendrites of the output pyramidal neuron...

The first application of electron detachment dissociation (EDD) to carbohydrates is presented. The structural characterization of glycosaminoglycan (GAG) oligosaccharides by mass spectrometry is a longstanding problem because of the lability of these acidic, polysulfated carbohydrates. Doubly-charged negative ions of four GAG tetrasaccharides are examined by EDD, collisionally activated dissoci...